Ch.

31 Hematologic Problems

ANEMIA
Definition and Classification
 ANEMIA IS NOT A DISEASE; it is a manifestation of a pathologic process

 Definition of Anemia
1) deficiency in the number of erythrocytes (RBCs)
2) The quantity of hemoglobin
3) The volume of packed RBCs (hematocrit)
 Anemia is identified by
o thorough history,
o physical examination,
o Labs of CBC, reticulocyte count, and peripheral blood smear.

 Various types of anemia

1) Morphologic classification (cellular characteristic)
 Based on descriptive, objective laboratory information about erythrocyte size and
color.
2) Etiologic classification (underlying cause)
 Related to clinical conditions causing the anemia, such as blood loss, decreased
erythrocyte production, or increased erythrocyte destruction.
 1) Impaired or a. Decreased  iron deficiency
decreased hemoglobin  Thalassemias (decreased globin synthesis) –
erythrocyte synthesis high incidence among African Americans
production  Sideroblastic anemia
b. Defective  Cobalamin (vit B12) deficiency
DNA  Folic acid deficiency
synthesis
c. Decreased  Aplastic anemia
number of  Anemia of myeloproliferative diseases (ex;
erythrocyte leukemia) and myelodysplasia
precursors  Chronic diseases or disorders
 Decreased erythropoietin in the kidneys
d. Chemotherap
y
2) Blood Loss a. acute  trauma
 blood vessel rupture
b. chronic  gastritis
 menstrual flow
 hemorrhoids
3) Increased a. Intrinsic  Abnormal hemoglobin (Hb S-Sickle cell
destruction of anemia) – high incidence among African
erythrocytes. Americans
 Enzyme deficiency (G6PD)
 Membrane abnormalities (paroxysmal
nocturnal hemoglobinuria, hereditary
spherocytosis)
b. Extrinsic  Physical trauma (prosthetic heart valves,
extracorporeal circulation, cardiopulmonary
bypass)
 Antibodies (isoimmune and autoimmune)
 Infectious agents, medications, and toxins
(malaria)
 Although the morphologic system is the most accurate means of classifying anemias, it is
easier to discuss patient care by focusing on the etiology of the anemia.

Clinical Manifestations
 Caused by body’s response to tissue hypoxia
 Specific manifestations can vary depending on the
1. Rate at which it has evolved
2. The presence of coexisting disease
3. Severity of the anemia – which is often determined by Hemoglobin (Hb) levels

 Clinical manifestations of anemia (table 31-3)

 Body Mild Moderate Severe
system (Hb 10 to (Hb 6 to 10 (Hb less than 6 g/dl)
14g/dl) g/dl)
Integument None None Pallor – results from ↓amounts of Hb and ↓blood flow
to the skin.
Jaundice* - occurs when hemolysis of RBCs results in an
↑concentration of serum bilirubin. (assess skin, sclera
of eyes, and mucous membranes for jaundice. They
reflect integumentary changes more accurately,
especially in dark-skin individual)
Pruritus* - occurs b/c of ↑serum and skin bile salt
concentrations.
Eyes None None Icteric conjunctiva and sclera* - assess for jaundice.
Retinal hemorrhage
Blurred vision
Mouth None None Glossitis, smooth tongue
Cardiovasc Palpitations ↑palpitations, Tachycardia, ↑pulse pressure,
ular “bounding systolic murmurs and bruit – due to low viscosity of
pulse” blood
Intermittent claudication,
angina, HF, MI
Pulmonary Exertional Dyspnea Tachypnea, orthopnea, dyspnea at rest
dyspnea
Neurologic None “Roaring in the HA, vertigo, irritability, depression, impaired thought
ears” processes
Gastrointesti None None Anorexia, hepatomegaly, splenomegaly, difficulty
nal swallowing, sore mouth
Musculoskel None None Bone pain
etal
General None Fatigue Sensitivity to cold, weight loss, lethargy
*Caused by hemolysis

• MILD STATES of anemia

o may be asymptomatic
o If patient pt has an underlying disease OR experience a compensatory response to heavy
exercise, symptoms develop and includes palpitations, dyspnea, and diaphoresis
• MODERATE STATES of anemia
o Cardiopulmonary symptoms are increased and pt may experience them while resting, as
well as activity
• SEVERE anemia
o has many manifestations including multiple body systems

• Cardiopulmonary Manifestations of severe anemia

o results from additional attempts by the heart and lungs to provide adequate amounts of
oxygen to the tissues
o Low viscosity of blood – develops systolic murmurs and bruits
o If myocardial O2 needs cannot be met, angina, MI may occur
o HF, cardiomegaly, pulmonary and systemic congestion, ascities, and peripheral edema
may develop if heart is overworked for an extended period of time

Nursing Management for Anemia

Nursing assessment
 Subjective
 Objective
o General – lethargy, apathy, general lymphadenopathy, fever
 o Integumentary – pale skin and mucous membranes; blue, pale white, or icteric sclera;
cheilitis’ poor skin turgor; brittle, spoon-shaped fingernails; jaundice; petechiae;
ecchymoses; nose or gingival bleeding; poor healing; dry, brittle, thinning hair
o Respiratory – tachypnea
o Cardiovascular – tachycardia, systolic murmur, dysrhythmias; postural hypotension,
widened pulse pressure, buirts (especially carotid); intermittent claudication, ankle
edema
o Gastrointestinal – hepatosplenomegaly; glossitis; beefy, red tongue; stomatitis;
abdominal distention; anorexic
o Neurologic – HA, roaring in the ears, confusion, impaired judgment, irritability, ataxia,
unsteady gait, paralysis, loss of vibration sense
o Possible findings - ↓RBCs, ↓ Hb, ↓Hct, ↑or↓ reticulocytes, MCV, serum iron, ferritin,
folate, or cobalamin (vit B12); heme (Guaiac)-positive stools; ↓serum erythropoietin
level, ↑or↓ LDH, bilirubin, transferrin

Nursing Diagnosis
 Activity intolerance related to weakness and imbalance between oxygen supply/demand as
evidenced by increased pulse and blood pressure in response to activity and patient report
of weakness
 Altered nutrition: less than body requirements related to inadequate nutritional intake and
anorexia as evidenced by weight loss, low serum albumin, decreased iron levels, vitamin
deficiencies
 Ineffective management of therapeutic regimen related to lack of knowledge about
appropriate nutrition and medication regimen as evidenced by questioning about lifestyle
adjustments, diet, and medications.

Planning
1) Assume normal activities of daily living
2) Maintain adequate nutrition
3) Develop no complications related to anemia

Nursing Implementation
• GOAL – correcting the cause of the anemia
• Numerous causes of anemia necessitate different nursing interventions specific to the
needs of the patient.
• Acute interventions
o Blood or blood product transfusion
o Drug therapy (ex; erythropoietin, vitamin supplements)
o Volume replacement
o Oxygen therapy to stabilize the patient
• Ongoing interventions
o Dietary and lifestyle changes (described with specific types of anemia) can reverse
some anemias.
 Ongoing assessment of patient’s knowledge regarding adequate nutritional
intake and compliance with safety precautions to prevent falls and injury and
drug therapies.

Gerontologic Considerations of Anemia

• Changes in RBC mass
• Decreased production of androgens
o Decline of Hb (about 1 g/dl) between ages 70 and 88 years in healthy men
o Minimal decline of Hb (about 0.2 g/dl) between ages 70 and 88 years in healthy
women
 • Cobalamin (vit 12) deficiency due to pernicious anemia, insufficient dietary intake,
malabsorption
• Multiple comorbid conditions in older adults
• Signs and symptoms include pallor, confusion, ataxia, fatigue, worsening angina, and
HF
 May go unrecognized in older adults b/c manifestations of anemia may be mistaken as
normal aging changes or overlooked b/c another health problem.

Anemia Caused by Decreased Erythrocyte Production (↓

erythropoiesis)
 Equilibrium between RBC production (erythropoiesis) and RBC destruction/loss
 Normal life span of RBC is 120 days (around 4 months)
 Three alterations in erythropoiesis
1) Decreased Hb synthesis may lead to iron-deficiency anemia, thalassemia, and
sideroblastic anemia
2) Defective DNA synthesis in RBCs (ex; cobalamin (vit 12) deficiency, folic acid deficiency)
may lead to megaloblastic anemias
3) Diminished availability of erythrocyte precursors may result in aplastic anemia and
anemia of chronic disease.

Iron-Deficiency Anemia

 One of the most common chronic hematologic disorders

 Found up to 30% of world’s population
 In US, occurs about 5 to 10% of people >45years
 Most susceptivle to iron-deficiency anemia
a. Very young
b. Those on poor diets
c. Women in their reproductive years.
 1 mg iron lost daily through feces, sweat, and urine in adult male and 1.5 mg/day in
normal menstruating women
 Median total iron loss with pregnancy is about 500 mg, or almost 2mg/day over 280 days
of gestation.

Etiology
1) Dietary irons may be inadequate for those who have higher iron needs such as
menstruating or pregnant women.
2) Malabsorption of iron may occur after GI surgery (removal or bypass of duodenum)
and malabsorption syndromes (disease of duodenum in which the absorption surface is
altered or destroyed) – iron absorption occurs in the duodenum
3) Blood loss – major cause of iron deficiency in adults.
• 2 mL of whole blood contain 1 mg of iron.
• Major sources of chronic blood loss are from GI and GU systems
• GI blood loss
o often not apparent (not easily seen).
o Loss of 50 to 75 ml of blood from upper GI tract –stools appear black (melena)
– black color results from iron in RBC
o Common cause of BI bleed – peptic ulcer, gastritis, esophagitis, diverticuli,
hemorrhoids, and neoplasia
• GU blood loss–
 o Average monthly menstrual blood loss is about 45 ml : loss about 22 mg of iron
o Common cause
 menstrual bleeding
 post menopauseal bleeding
4) pregnancy - due to diversion of iron to the fetus for erythropoiesis, blood loss at
delivery, and lactation
5) chronic renal failure and dialysis treatment may induce iron-deficiency anemia b/c
of the blood lost in dialysis equipment
6) frequent blood sampling

Manifestations
• In early course – pt. may be asymptomatic
• When chronic – any general manifestations of anemia may develop
• Specific clinical symptoms of iron deficiency anemia – caused by lack of iron in the tissues
o Pallor – most common findings
o Glossitis (tongue inflammation) – 2nd most common
o Cheilitis (lips inflammation)
o Patient may report HA, paresthesias, burning sensation of the tongue

Diagnostic Studies
• Specific diagnostic studies to determine the cause of iron deficiency anemia
o Stool guaiac test - test for unseen blood in stool
o Endoscopy and colonoscopy – to detect GI bleeding
o Bone marrow biopsy – if other tests are inconclusive
• LABs
Hb/Hct MCV Reticulocyt Serum TIBC Transferr Ferritin Bilirubin
es Iron in
Iron ↓ ↓ Normal or ↓ ↑ N or ↓ ↓ N or ↓
deficiency slight ↓ or
↑

Collaborative Care
• GOAL – treat the underlying disease that is causing reduced intake (ex; malnutrition,
alcoholism) or absorption of iron.
• TREATMENT – efforts are directed toward replacing iron.
o TEACH which foods are good sources of iron (Nutrition)
o If nutrition is already adequate  use oral or parenteral iron supplements
o From acute blood loss  transfusion of packed RBCs
• Drug Therapy
o Oral iron should be used whenever possible. (inexpensive and convenient)
o Consideration in iron administration (5 factors)
1. NO EC or SR capsules.
-b/c iron is absorbed best from duodenum and proximal jejunum. EC or SR
release farther down in the GI tract.(counter productive and expensive)
2. Daily dose of 150 to 200 mg of iron.
3. Iron should be taken about an hour before meals, when duodenal mucosa is most
acidic.
-b/c iron is best absorbed as ferrous sulfate Fe2+ in an acidic environment.
Avoid binding the iron with food. Take iron with vit C or orange juice  enhances
absorption. Due to GI side effects, may need to ingest iron with meals.
4. Liquid forms should be diluted and ingested through a straw.
-b/c undiluted liquid iron may stain patient’s teeth.
5. Adjust dose and type of iron supplement if side effects develop
  Ex) many individuals who need supplemental iron cannot tolerate ferrous
sulfate b/c of the effects of the sulfate base  ferrous gluconate may be an
acceptable substitute.
 TEACH – stools may turn black b/c GI excretes iron
 GI side effects
 Heartburn,
 Constipation (common) –patient should be started on stool softeners
and laxatives when started on iron
 Diarrhea
o Parenteral use of iron may be necessary (IV or IM)
 For malabsorption, intolerance of oral iron, a need for iron beyond oral limits, or
poor pt. compliance in taking oral preparations of iron.
 Iron-dextran complex (INFeD) contains 50 mg/ml of elemental iron in 2 mL – longest
use historically.
 Alternatives : Sodium ferrous gluconate and iron sucrose– may provide less risk of
life-threatening anaphylaxis and delayed serum sickness
 TEST DOSE of parenteral iron often done to assess potential allergic reaction
 IM
• May stain skin – so use separate needles for withdrawing the solution and
for injecting medication
• Leave approx. 0.5 ml of air in synringe to completely clear iron from
syringe
• Give deep IM in upper outer quadrant of buttocks
• Use 2 to 3 inch, 19 to 20 gauge needle
• No more than 2 ml of iron given in single injection
• Z-track technique to prevent leackage to subq
• No massage after injection
 IV
• Iron dextran should not be mixed with other meds or added to parenteral
nutrition solutions
• Give undiluted
• Rate should be no more than 1 ml/min
• IV line should be flushed with normal saline.

Nursing Management for Iron-Deficiency Anemia

 Recognize groups of individuals who are at risk - premenopausal women, pregnant
women, persons from low socioeconomic backgrounds, older adults, and individuals
experiencing blood loss.
 TEACHING – diet (foods high in iron)
 Supplemental iron important for pregnant women
 Discuss patient needs for diagnostic studies to identify cause
 Hb and RBC counts are reassessed to evaluate response to therapy
 Compliance with dietary and drug therapy is emphasized
 Patient needs to continue to take iron therapy for 2 to 3 months after Hb levels returns
to normal to replenish body’s iron stores
 MONITOR potential liver problems related to iron storage for those who require lifelong
iron supplementation.

Thalassemia

Etiology
  Thalassemia is a group of diseases that have an autosomal recessive genetic basis
involving inadequate production of normal hemoglobin. (abnormal Hb synthesis)
 Hemolysis also occurs in thalassemia
 But insufficient production of normal Hb is the predominant problem.
 Thalassemia is due to an absent or reduced globulin protein
• α-thalassemia – absent or reduced α-globin chains
• β-thalassemia – absent or reduced β-globin chains
 commonly found in members of ethnic groups whose origins are near the Mediterranean
Sea and equatorial or near-equatorial regions of Asia, the Middle East, and Africa
 person with thalassemia may have a heterozygous or homozygous form of dz.
• thalasemia minor (or thalassemic trait) –person who is heterozygous has one
thalassemic gene and one normal gene
• thalassemia major – a homozygous person has two thalassemic genes, causes severe
condition

Manifestations
• Thalassemia major
o life threatening disease in which growth (both physical and mental) is retarded.
o Pale and displays other general symptoms of anemia.
o Symptoms develop in childhood by 2 years of age
o Pronounced splenomegaly and hepatomegaly
o Jaundice – from RBC hemolysis
o Blood cell production is stimulated bone marrow becomes packed with immature
erythroid precursors that die further erythopoiesis stimulated Chronic bone marrow
hyperplasia and expansion of the marrow space  causes thickening of the cranium and
maxillary cavity.
• Thalassemia minor
o Frequently asymptomatic
o Mild to moderate anemia
o Patient has microcytosis (small cells) and hypochromia (pale cells)

Diagnostics
Labs
Hb/Hct MCV Reticulocyt Serum TIBC Transferr Ferritin Bilirubi
es Iron in n
Thalassemi ↓ N or↓ ↑ ↑ ↓ ↓ N or ↑ ↑
a major

Collaborative Care
• No specific drug or diet therapies
• Thalassemia minor
o Requires no treatment b/c body adapts to the reduction of normal Hb
• Thalassemia major
• Managed with blood transfusion or exchange transfusions in conjunction with IV
deferoxamine (Desferal)
o deferoxamine(Desferal) is a chelating agent that binds to iron to reduce the iron
overloading (hemochromatosis) that occurs with chronic transfusion therapy.
 S/E: visual blurring, hearing loss, tinnitus, and knock-knees
o Keep the Hb level at approximately 10 g/dl – low enough to maintain pt’s own
erythropoiesis without enlarging the spleen.
o Zinc supplementation may be needed – it is reduced with chelation therapy
 o Ascorbic acid supplementation during chelation therapy – increases urine excretion
of iron (should not be taken..otherwise,,as it increases the absorption of dietary
iron)
o NO iron supplements
o May be treated by splenectomy since RBCs are sequestered in the enlarged spleen.
o Hep C is present in majority of pts older than 25 years b/c of receiving blood
transfusion before screening for hep C virus.
 Hep C may result in cirrhosis and hepatocellular carcinoma
o Cardiac complications from iron overload are reported to cause 71% of the deaths.
o Pulmonary dz and HTN also contribute to early death
o MONITOR hepatic, cardiac, pulmonary organ function
o Endocrinopathies (hypogonadotrophic hypogonadism) and thrombosis may also be
complications

Megaloblastic Anemias
 Group of disorders caused by impaired DNA synthesis
 Megaloblasts – abnormal and large RBCs (macrocytic) – macrocytic RBCs are easily
destroyed due to fragile cell membranes
 Causes
• cobalamin (vit B12) deficiency - common
• Folic acid deficiency - common
• Suppression of DNA synthesis by drugs
• Inborn errors of cobalamin and folic acid metabolism
• Erythroleukemia – malignant blood disorder characterized by a proliferation of
erythropoietic cells in bone marrow
Cobalamin (Vitamin B12) Deficiency
 Parietal cells of the gastric mucosa normally secretes a protein called intrinsic factor (IF). IF
is required for cobalamin (extrinsic factor) absorption in the distal ilium. If IF is not secreted,
cobalamin will not be absorbed.
 Causes of cobalamin deficiency
• Dietary deficiency
• Deficiency of gastric intrinsic factor
o Pernicious anemia – most common cause
 A disease in which the gastric mucosa is not secreting IF because of antibodies
being disrected against the gastric parietal cells and/or IF itself
 Insidious onset that begins in middle age or later (usually after age 40) with 60
years being the most common age at diagnosis
 Occurs frequently in persons of Northern European ancestry (particularly
Scandinavians) and African Americans(disease begins early with higher
frequence in women and often severe)
o Gastrectomy
• Intestinal malabsorption
• Increased requirement
• Chronic alcoholism

Etiology
1. Cobalamin deficiency results from loss of IF-secreting gastric mucosal cells or
impaired absorption of cobalamin in the distal ileum
o GI surgery such as gastrectomy
o Pts who have had a small bowel resection involving the ileum
 oPatients with Crohn’s disease, ileitis, diverticuli of small intestine, chronic atrophic
gastritis
2. Long-term users of H2-histamine receptor blockers
3. Pernicious anemia is caused by absence of IF from either gastric mucosal atrophy or
autoimmune destruction of parietal cells – results in decrease of hydrochloric acid
secretion by the stomach (acidic environment is required for IF secretion)

Clinical Manifestations
• General symptoms (table 31-3)
• GI manifestations – sore tongue, anorexia, nausea, vomiting, and abdominal pain
• Typical Neuromuscular manifestations – weakness, paresthesias of feet and hands,
reduced vibratory and position senses, ataxia, muscle weakness, impaired thought
processes (confusion, dementia)
• Insicious onset – may take several months for manifestations to develop

Diagnostics
• Labs
Hb/Hc MC Reticulocyt Seru TIB Transferri Ferriti Bilirubi Seru Cobalami Hycrochlori
t V es m C n n n m n c acid
Iron folate
Cobalami ↓ ↑ N or ↓ N or N Slight ↑ ↑ N or norm low decreased
n ↑ slight al
deficienc ↑
y

• RBCs appear large (macrocytic) and have abnormal shapes

• Serum cobalamin levels are reduced
• If serum folate levels are normal and cobalamin levels are lowmegaloblastic anemia is
due to a cobalamin deficiency
• Patients with pernicious anemia are potential for gastric cancer – gastroscopy and biopsy
of gastric mucosa may be done
• Schilling test – assessing parietal cell function
o Administer radioactive cobalamin measure amount of cobalamin excreted in the
urine
o Who CANNOT absorb cobalamin excretes only a SMALL amount of this radioactive
form.
• Follow a parenteral administration of IF
o Absorption of cobalamin when IF is added is diagnostic of pernicious anemia
• Serum methylmalonic acid (MMA) and serum homocysteine can be used if other tests are
not definite.

Collaborative Care
• Increasing dietary cobalamin does not correct the anemia – b/c not able to absorb
cobalamin if IF is lacking or imapred absorption in the ileum (but still…good nutrition is
required)
• TREATMENT of CHOICE - Parenteral (cyanocobalamin or hydroxocobalamin) or intranasal
(Nascobal) administration
• Without administration  die in 1 to 3 years
• Dosage and frequency may vary – typical treatment schedule consists of 1000mg of
cobalamin IM daily for 2 weeks weekly until hematocrit is normalmonthly FOR LIFE
• High dose oral and sublingual cobalamin available
• Anemia can be REVERSED if supplemental cobalamin is used. May be irreversible if one
has had long-standing neuromuscular complications
Folic Acid Deficiency
• Also causes megaloblastic anemia (large RBCs with abnormal shapes)
• Folic acid is required for DNA synthesis leading to RBC formation
• Causes are
1. Poor nutrition -lack of leafy green vegetables, liver, citrus fruits, yeast, dried beans, nuts,
and grains
2. Malabsorption syndroms- small bowel disorders
3. Drugs that impede the absorption and use of folic acid(ex; methotrexate), antiseizure
drugs (ex; Phenobarbital, diphenylhydantoin (Dilantin), and others (ex; trimethoprim,
sulfasalazine)
4. Alcohol abuse and anorexia
5. Hemodialysis patients - folic acid is lost during dialysis

Manifestations
• Similar with cobalamin deficiency
• Develops insidiously
• GI disturbances include dyspepsia and a smooth, beefy red tongue
• ABSENCE OF NEUROLOGIC PROBLEMS is an important diagnostic finding – differentiates folic
acid deficiency from cobalamin deficiency.
• Labs

Hb/H MC Reticulocy Seru TIB Transfer Ferriti Bilirub Seru Cobala Hycrochlo
ct V tes m C rin n in m min ric acid
Iron folat
e
Folic ↓ ↑ N or ↓ N or N Slight ↑ ↑ N or low normal positive
acid ↑ slight
deficien ↑
cy
*Serum folate (normal is 3 to
25 mg/ml)
Collaborative Care
• Treatment – replacement therapy
• Usual dose is 1mg /day by mouth
• In malabsorption states, up to 5 mg/day may be required
• Duration depends on reason for deficiency
• Encourage to eat foods containing large amounts of folic acid

Nursing Management for Megaloblastic Anemia

• ATTENTION of signs and symptoms of possible megaloblastic anemias
• Early detection may REVERSE symptoms, (although dz development cannot be prevented) –
so those with a positive family history of pernicious anemia should be evaluated (b/c
familial predisposition)
• NCP 31-1
• Ensure that injuries are not sustained b/c diminished sensatiosn to heat and pain resulting
from neurologic impairment – cobalamin deficiency. Protect from falls, burns, and trauma. If
heat therapy required assess skin at frequent intervals to detect redness
• Ongoing care – compliance with treatment
• Careful follow-up to assess for neurologic difficulties
• POTENTIAL FOR CANCER may be increased (ex; pts with atrophic gastritis-related pernicious
anemia gastric carcinoma, alcohol increasesoral and esophageal cancer)

Anemia of Chronic Disease

Etiology
 Chronic inflammatory, autoimmune, infectious, or malignant disease can lead to anemia of
chronic disease
 Associated with an underproduction of RBCs and mild shortening of RBC survivial.
 RBCs are normocytic, normochromic, and hypoproliferative
 Mild anemia,
 Can be more severe if primarily immune driven. Released cytokines causes an increased
uptake and retention of iron within macrophages diversion of iron from circulation into
storage siteslimitation of iron availability for erythroid progenitor cells, and iron restricted
erythropoiesis
 Renal disease –
o Primary factor causing anemia is decreased erythropoietin
 Any condition that causes ↑RBC destruction (autoimmune hemolysis) + failure to augment
erythropoiesis = anemia
 Myelosuppression and ↓erythropoiesis caused by disease, medications (chemotherapy), or
radiation will contribute to anemia
 Human immunodeficiency virus(HIV) and its treatment, hepatitis, malaria, and bleeding
episodes
 Hypopituitary and hypothyroid states - leads to reduced tissue metabolism tissue oxygen
needs are diminished ↓production of erythropoietin by the kidneys.
 Adrenalectomy or Addison’s disease results in anemia due to adrenal dysfunction

Labs
Hb/H MCV Reticulocy Seru TIB Transfer Ferriti Bilirubi Seru Cobalamin
ct tes m C rin n n m
Iron folate
Chroni ↓ N or↓ N or ↓ N or ↓ N or ↓ N or↑ N norm normal
c ↓ al
diseas
e

Collaborative Care
• Must FIRST BE RECOGNIZED and DIFFERENTIATED from other anemias
• Best TREATMENT – correction of underlying disorder
• If severe – blood transfusion may be indicated, but are not recommended for long term
• Anemia related to renal disease or anemia related to cancer therapies – use Erythropoietin
therapy (Epogen, Procrit). Darbepoetin (Aranesp) – newer form of erythropoietin, longer
duration of action than erythropoietin
• IV iron should ONLY be administered IF NECESSARY to improve erythropoietin therapy.

Aplastic Anemia
 Disease in which patient has peripheral blood pancytopenia (↓ of all blood cell types – RBCs,
WBCs, and platelets) and hypocellular bone marrow.

Etiology
 Low incidence; 4:1million
 Two major classification
1) Congenital origin – caused by chromosomal alterations. Approx. 30% of the aplastic
anemias appear in childhood are inherited
o Fanconi syndrome
o Congenital dyskeratosis
o Amegakaryocytic
o Thrombocytopenia
 o Schwachman-Diamond Syndrome
2) Acquired aplastic anemia –
o results from exposure to ionizing radiation
o chemical agents (ex; benzene, insecticides, arsenic, alcohol)
o viral and bacterial infections (ex; hepatitis, parvovirus, biliary tuberculosis),
o Prescribed medications (ex; alkylating agents, antiseizure agents,
antimetabolites, antimicrobials, gold)
o Pregnancy
o Idiopathic

Manifestations
 Present abruptly(over days) or insidiously (over weeks to months)
 Vary from mild to severe
 Symptoms caused by suppression of any or all bone marrow elements
 Fatigue and dyspnea, cardiovascular, and cerebral responses, may be seen
 Nutropenia patients (low neutrophil count) – susceptible to infection, febrile
 Thrombocytopenia – predisposition to bleeding (ex; petechiae, ecchymosis, epistaxis)

Diagnostic Studies
 Labs
Hb/H MCV Reticulocyt Seru TIBC Transferr Ferriti Bilirub WB platel Bleedin
ct es m in n in C et g time
Iron
Aplasti ↓ N or ↓ N or N or N N N low low prolong
c slight ↑ ↑
anemi ↑
a

 Hb, WBC, platelet values are often decreased due to affected all marrow elements. Other
RBC indices are generally normal.(normocytic, normochromic anemia)
 Reticulocyte count is low
 Prolong bleeding time
 ↑serum iron and ↑total iron-binding capacity(TIBC) - initial signs of erythropoiesis
suppression
 Bone marrow biopsy, aspiration, and pathologic examination may be done
 Because the marrow is hypocellular with increased yellow marrow (fat content), findings are
very important .

Collaborative Care
 medical management
o hematopoietic stem cell transplant (HSCT)
o immunosuppressive therapy with antithymocyte globulin (ATG) – horse serum that
contains polyclonal antibodies against human T cells. Can cause anaphylaxis and
serum sickness. RATIONALE – aplastic anemia is an immune-mediated disease
o cyclosporine or high dose cyclophosphamide (Cytoxan)
 TREATMENT OF CHOICE is an HSCT
o For those who are <45 yrs who do not respond to immunosuppressive therapy
o who have a human leukocyte antigen (HLA) – matched donor
o Best results – younger pts, who did not have previous blood transfusions b/c prior
transfusions increase risk of graft rejection
 TREATMENT OF CHOICE is an Immunosuppression with ATG or cyclosporine or high-dose
cyclophosphamide
o For older adult without an HLA-matched donor
Nursing Management for Aplastic Anemia
  Based on identifying and removing the causative agent and providing supportive care until
pancytopenia REVERSES
 See nursing interventions for
a) anemia NCP 31-1
b) thrombocytopenia NCP 31-2
c) neutropenia NCP 31-3
 nursing actions DIRECTED at PREVENTING COMPLICATIONS from INFECTIONS and
HEMORRHAGE
Anemia Caused by Blood Loss
Acute Blood Loss

 Result of sudden hemorrhage

 Causes
o Trauma
o Complications of surgery
o Conditions or disease that disrupt vascular integrity
 2 clinical concerns
1) Hypovolemic shock – sudden reduction in total blood volume
2) Body maintaining its blood volume by slowly increasing plasma volume. This occurs
when acute loss is more gradual. Although circulating fluid volume is preserved, # of
RBCs available to carry oxygen is significantly diminished

Manifestations
 Manifestations are caused by body’s attempts to maintain adequate blood volume and meet
oxygen requirements.
 Understand clinical signs and symptoms of patient are more important than the lab
values.
o Ex) adult with peptic ulcer bleeding has 750 ml hematemesis (15% of normal total
blood vlume) within past 30 minutes , may have postural hypotension. BUT have
normal Hb and Hct values.
 Be alert to patient’s expression of pain. Internal hemorrhage may cause pain due to
tissue distention, organ displacement, and nerve compression. Localized pain or referred
pain.
o Ex) retroperitoneal bleeding- patient may not have abdominal pain. But may have
numbness and pain in lower extremity secondary to compression of lateral cutaneous
nerve, located in the first to third lumbar vertebrae
 Major complications - shock

Diagnostic studies
-When acute blood loss, values may seem NORMAL OR HIGH FOR 2 TO 3 DAYS because plasma
volume has not yet had a chance to increase. Therefore, loss of RBCs is not showed in the lab.

-However, ONCE PLASMA IS REPLACED by endogenous and exogenous means, RBC mass is less
concentrated.  lab will reflect the blood loss by showing ↓RBC, ↓Hb, ↓Hct.

Hb/H MCV Reticulocyt Seru TIBC Transferr Ferriti Bilirub RBC

ct es m in n in
Iron
Aplasti ↓ N or ↓ N or ↑ N N N N N low
c
anemi
a
*The blood for this lab results is drawn 2 to 3 days after acute blood loss.
Collaborative Care
1) Replacing blood volume to prevent shock
2) Identify the source of hemorrhage and stop the blood loss

• IV fluids used in emergencies

o Dextran, hetastarch, albumin, and/or crystalloid electrolyte solutions such as
Lactated Ringers.
• Once replaced the volume, CORRECT RBC LOSS!! Blood transfusions (packed RBCs)
may be needed b/c it takes 2 to 5 days for the body to make RBCs. If bleeding is related to
platelet or clotting disorder, replace those that are deficient.
• May need IRON supplement (iron affects the marrow production of erythrocytes). If
anemia still exists after the acute blood, give oral or parenteral iron b/c dietary iron is not
adequate.

Nursing Management for Acute Blood Loss

• Postoperative Patients – MONITOR blood loss from drainage tubes and dressings and
implement appropriate actions
• NCP for pt with anemia resulting from acute blood loss  most likely blood transfusion

 There should be NO NEED for LONG TERM TREATMENT for this for acute blood loss
anemia.

Chronic Blood Loss

• Usually related to depletion of iron stores usually considered as iron-deficiency anemia.
(ex; bleeding ulcer, hemorrhoids, menstrual and postmenopausal blood loss)
• Labs
Hb/H MCV Reticulocyt Seru TIBC Transferr Ferriti Bilirub
ct es m in n in
Iron
Aplasti ↓ N or ↓ N or ↓ N or ↓ N or ↓ N or ↑ N
c ↓
anemi
a

MANAGEMENT
• Identify source! STOP THE BLEEDING
• Supplemental IRON may be required.
• NCP 31-1

Anemia Caused by Increased Erythrocyte Destruction

Hemolytic Anemia
• 3rd major cause of anemia
• Caused by destruction of hemolysis of RBCs at a rate that exceeds production

Causes of hemolysis
• Intrinsic hemolytic anemias (heredity) – results from RBC defects caused by
o Sickle cells (abnormal Hb)
o Enzyme deficiencies that alter glycolysis (glucose-6 phosphate dehydrogenase
[G6PD] deficiency)
 o RBC membrane abnormalities
• Extrinsic hemolytic anemias (acquired) – RBCs are normal but damaged by external
factors such as
o Trapping of cells within the sinuses of the liver or spleen
o Antibody-mediated destruction
o Toxins
o Mechanical injury (ex; prosthetic heart valves)

Two sites of hemolysis

• Intravascular hemolysis – occurs within the circulation
• Extravascular hemolysis – takes place in the macrophages of the spleen (primary site of
destruction of old RBCs), Liver, and bone marrow

• Manifests general symptoms of anemia and clinical manifestations specific to the type of
anemia
• JAUNDICE – ↑bilirubin levels due to ↑destruction of RBCs
• Splenomegaly, hepatomegaly due to hyperactivity
• MAJOR FOCUS OF TREATMENT – MAINTAIN RENAL FUNCTION (when RBCs are hemolyzed,
Hb molecule is released and filtered by kidneys. Hb molecules may accumulate and
obstruct renal tubles  lead to Acute tubular necrosis
Sickle Cell Anemia
 Group of inherited, autosomal recessive disorders
 Mutation in β–globin gene located on chromosome 11
 Presence of an abnormal form of hemoglobin, Hemoglobin S (Hb S) in the erythrocyte
Hemoglobin S – involves substitution of VALINE for glutamic acid on the β-globin chain of
Hb. Hb S causes erythrocyte to stiffen and elongate taking on a sickle shape in response to
low oxygen levels.

 Identified during infancy or early childhood.

 Incurable
 Fatal by middle age from renal and pulmonary failure

 Predominant in African Americans (400:1)

Also affects people of Mediterranean, Caribbean, South and Central American, Arabian, or
East Indian ancestry.

Etiology and Pathophysiology

Types of sickle cell disease
1) Sickle cell anemia – homozygous for Hb S (Hb SS)
a. Inherits Hb S from both parent
b. Most severe of SCD syndromes
2) Sickle cell thalassemia
a. Inherits Hb S from one parent and another abnormal Hemoglobin (thalassemia)
from other parent.
b. Less common and less severe
3) Sickle cell Hb C dz
a. Person inhereits Hb S from one parent and another abnormal Hemoglobin
(Hemoglobin C) from other parent.
b. Less common and less severe
4) Sickle cell trait – heterozygous for hemoglobin S (Hb AS)
a. Person has inherited Hb S from one parent and normal Hb (Hb A) from other parent
b. Very mild to asymptomatic condition
Sickling Episodes
• Major pathology is the sickling of RBCs.
-Most commonly triggered by low oxygen tension in the blood (hypoxia or
deoxygenation of RBCs) and can be caused by viral or bacterial infection(most common
factor), high altitude, emotional or physical stress, surgery, blood loss, dehydration, increased
hydrogen ion concentration (acidosis), increased plasma osmolality, decreased plasma
volume and low body temperature. Can also occur without an obvious cause.
-Hemostasis promotes more local hypoxia, deoxygenation of more erythrocytes, and more
sickling  hemolyzed by the spleen, leading to anemia

• Sickled RBCs become rigid, elongated, drescent shape. Cannot easily pass through
capillaries, small vessels, and can cause vascular occlusion, leading to acute or chronic
tissue injury

• Sickling of cells is reversible with reoxygenation, BUT it EVENTUALLY becomes irreversible b/c
recurrent sickling causes cell membrane damage.

• CLINICAL HALLMARKS – vasoocclusive phenomena and hemolysis

• Sickle cell crisis – severe, painful, acute exacerbation of RBC sickling causing a
vasoocclusive crisis.
-Blood flow impaired, vasospasm occurs, further restricting blood flow
-Severe capillary hypoxia causes changes in membrane permeability, leading to plasma
loss, hemoconcentration, development of thrombi, further circulatory stagnation, tissue
ischemia, infarction, and necrosis, and SHOCK (life threatening consequence of sickle cell
crisis)
-Can begin suddenly and persist for days to weeks

• Sickling episodes are unpredictable with frequency, extent, and severity

-Largely dependent on the percentabe of Hb S present (Hb SS is most severe form due to high
percentage of Hb S)

Manifestations
• Effects vary from person to person
• Many people are in good health the majority of the time. But they may have chronic health
problems and pain b/c of organ tissue hypoxia and damage (ex; kidneys and/or liver)
• May be anemic but asymptomatic except during sickling episodes.
• Manifestations –
o PAIN – primary symptom, especially during sickle cell crisis b/c of ischemia of
tissue. Can affect any area of body or several sites simultaneously (back, chest,
extremities, abdomen are most common)
o Grayish cast skin
o Pallor of mucous membranes – most sickle cell anemic pts have dark skin. So
examine mucous membranes for pallor.
o Fatigue
o Decreased exercise tolerance
o Jaundice (common)
o Prone to cholelithiasis (gallstones)
o ½ of episodes accompany fever, swelling, tenderness, tachypnea, HTN, nausea,
vomiting

Complications
• Spleen, lungs, kidneys and brain most often affected due to high oxygen needs
 -INFECTION (major cause of morbidity and portality) due to failure of the SPLEEN to
phagocytize foreign substances as it becomes infracted and dysfunctional (2 to 4 yrs of
age)
o Pneumonia (most common), aplastic crisis, hemolytic crisis, and gallstones
-Acute chest syndrome – acute pulmonary complications that include pneumonia, tissue
infarction, and fat embolism.
o Characterized by fever, chest pain, cough, pulmonary infiltrates, and dyspnea
-Pulmonary infarctions may cause pulmonary HTN, MI, HF, and cor pulmonale.
-Cardiomegaly leading to HF
-Autosplenectomy – spleen becomes small because of repeated scarring
-Retinal vessel obstruction  hemorrhage, scarring, retinal detachment, and blindness
-renal failure
-Stroke – thrombosis and infarction of cerebral blood vessels
-Osteoporosis and osteosclerosis after infarction
-Chronic leg ulcers prevalent around ankles due to hypoxia
-Priapism (persistent penile erection) if penile veins become occluded

Diagnostic Studies
 Peripheral blood smear – reveal sickled cells and abnormal reticulocytes.
 Sickling test – uses RBCs (in vitro) and exposes them to a deoxygenation agent
 Electrophoresis of hemoglobin readily identifies the abnormal Hb
 DNA testing - expensive
 Skeletal x-rays – bone and joint deformities and flattening
 MRI – diagnose a stroke caused by blocked cerebral vessels from sickled cells
 Doppler – assess DVT
 Chest x-ray – diagnose infection or organ malfunction
 Labs – findings of hemolysis (jaundice, elevated serum bilirubin levels)
Hb/H MCV Reticulocyt Seru TIBC Transferr Ferriti Bilirubi
ct es m in n n
Iron
Sickle cell ↓ N ↑ N or N or ↓ N N ↑
↑

Nursing and Collaborative Management for Sickle cell Disease

NO SPECIFIC TREATMENT for the disease
Care is directed toward
1) Alleviating symptoms from complications of the disease
2) Minimize end-organ damage

Acute chest syndrome –

1) treatment broad-spectrum antibiotics,
2) O2 therapy,
3) fluid therapy,
4) folic acid supplementation
5) NO IRON THERAPY.

Sickle cell crisis –

1) May require hospitalization
2) Oxygen – treat hypoxia and control sickling
3) Rest – to reduce metabolic requirements
4) Fluids and electrolytes – to reduce blood viscosity and maintain renal function
5) Transfusion therapy when aplastic crisis occurs – may require chelation therapy to reduce
transfusion – produced iron overload (like thalassemia major)
 6) PAIN – undertreatment is major problem due to lack of knowledge of health care
professionals
During acute crisis – continous large doses of opioid analgesics(not PRN) + PCA pump
Drugs of Choice – Morphine and hycromorphone
Contraindicated drugs – Meperidine (Demerol) - can cause seizure due to toxic
accumulation.
AFTER DISCHARGE, patients will often CONTINUE ON ORAL OPIOID ANALGESICS
7) Multimodal and multidisciplinary approach is often needed – NSAIDs, antineuropathic pain
meds (TCA, antiseizure meds), local anesthetics, nerve blocks, transelectrodermal nerve
stimulator (TENS), and acupuncture.

*DRUGS
Hydroxyurea (Hydrea): increases the production of hemoglobin F (fetal hemoglobin). Given in
low dose to minimize the risk of drug-induced cancer and leukemia. If ↑Hb F, then, ↑Hb
concentration, ↓sickle cells, and ↓in hemolysis.

*Surgery
Hemtaopoietic stem cell transplantation (HSCT) – only treatment that can CURE pts with
SCD but the use is limited.

*TEACH patient and family about the disease and the reasons for supportive care.
 Teach to avoid crisis: reduce the chance of developing hypoxia (avoiding high altitudes).
 Maintain adequate fluid intake
 Treat infections promptly: Seek medical attention quickly when having problems with the
upper respiratory tract infections.
 Educate pain control: minor pain without infection or without symptoms warranting
medical attention can sometimes be managed at home
 Occupational therapists and physiotherapisis : help achieve optimum physical functioning and
independence.
 psychologist may help cope with anxiety and depression
 Immunizations – pneumovax, Haemophilus influenza, influenza, and hepatitis
 Chronic leg ulcers – bed rest, antibiotics, warm saline soaks, mechanical or enzyme
debridement, grafting if necessary
 Priapism – pain meds and nifedipine (Procardia)-CCB
 AFTER DISCHARGE, patients will often CONTINUE ON ORAL OPIOID ANALGESICS.

Acquired Hemolytic Anemia

 Acquired Hemolytic Anemia is a destruction of RBCs from an extrinsic cause. PATIENT’S

RBCs ARE NORMAL. The destruction is caused by an extrinsic factor.

Extrinsic cause of hemolysis has 3 categories

1) Physical factor
 Results from the exertion of extreme force on the cells
o Traumatic events include hemodialysis, extracorporeal circulation used in
cardiopulmonary bypass, and prosthetic heart valves
o Force needed to push blood through abnormal vessels (burned or affected by
angiopathic disease such as DM)

2) Immune reactions (antibodies destroy RBCs by mechanism in antigen-antibody

reactions)
a. Isoimmune Reactions occur when antibodies develop against antigens. Recipient’s
antibodies hemolyze donor cells when blood transfusion
 b. Autoimmune reactions result when individuals develop antibodies against their own
RBCs.
i. May be idiopathic
ii. No prior hemolytic history as a result of the immunoglobulin IgG covering the
RBCs
iii. Or secondary to other autoimmune disease (SLE, erythromatosus), leukemia,
lymphoma, or medications (penicillin, indomethacin [Indocin],
phenylbutazone [Butazolidine], phenacetin, quinidine, quinine, and
methyldopa [Aldomet])

3) Infectious agents and toxins

a. Infectious agents foster hemolysis in four ways
i. By invading the RBC and destroying its contents (ex; parasites such as in malaria)
ii. By releasing hemolytic substances (ex; Clostridium perfringens)
iii. By generating an antigen-antibody reaction
iv. By contributing to splenomegaly as a means of increasing removal of damaged
RBCs from the circulation
b. Toxins may cause RBC hemolysis
Chemicals such as oxidative drugs, arsenic, lead, copper, and snake venoms

Labs
Hb/H MCV Reticulocyt Seru TIBC Transferr Ferriti Bilirubi
ct es m in n n
Iron
Hemolytic ↓ N or ↑ ↑ N or N or ↓ N N or ↑ ↑
anemia ↑

Treatment and management

 General supportive care until causative agent can be eliminated or at least rendered less
injurious to RBCs
 NURSE! Get ready to institute appropriate emergent therapy b/c HEMOLYTIC CRYSIS is a
potential consequence.
 May need corticosteroids and blood products or removing the spleen.

Hemochromatosis
 Hemochromatosis is an autosomal recessive disease. The characteristics of Increased
intestinal iron abosorption causes INCREASED TISSUE IRON DEPOSITION

Etiology
1) Primarily genetic
a. Most common genetic disorder among whites (3:5 per 1000 whites of European
ancestry)
b. Normal total body iron is 2 to 6g. hemochromatosis patients accumulate iron at a
rate of 0.5 to 1.0g/year = may exceed total iron concentrations of 50g
c. Symptoms usually develop between 40 to 60 years of age
2) Secondary to diseases such as thalassemia and sideroblastic anemia
3) Liver disease
4) Multiple blood transfusions

Manifestations
 Early symptoms – nonspecific , includes fatigue, arthralgia, impotence, abdominal pain, and
weight loss
 Later symptoms – liver enlargement and eventually cirrhosis b/c of excess iron accumulations
 Then, other organs become affected, resulting in DM, skin pigment changes
(bronzing), cardiac changes(cardiomegaly), arthritis, and testicular atrophy.

Diagnostic Studies
 Physical examinations – enlarged liver and spleen and pigment changes in skin
 Labs - ↑serum iron, ↑ TIBC, ↑ serum ferritin
Hb/Hc MC Reticulocy Serum TIBC Transferr Ferritin Bilirubin
t V tes Iron in
Hemochromato ↑ ↑ ↑
sis

 Molecular testing for KNOWN GENETIC MUTATIONS used to CONFIRM diagnosis. If not
definitive
 Liver biopsy –quantify amount of iron and establish the diagnosis

Collaborative Care
 GOAL – remove excess iron from body and minimize any symptoms the patient may have
-REMOVE 500 ml of blood each week for 2 to 3 years until iron stored in the body are
depleted. Less frequent afterwards.
-Organ involvement management
-Dietary modifications – avoid vit C, Iron supplements, uncooked seafood, and iron-rich
foods
-Most common cause of death are cirrhosis, liver failure, hepatic carcinoma, and cardiac
failure.

Polycythemia

 Polycythemia is the production and presence of INCREASED numbers of RBCs. Numbers

are so great that the blood circulation is impaired due to INCREASED BLOOD VISCOSITY
(hyperviscosity) and VOLUME (Hypervolemia).

Etiology and Pathophysiology

Two types of polycythemia
1. Primary polycythemia (or polycythemia vera)
-Chronic myeloproliferative disorder
-Caused by a chromosomal mutation in a single pluripotent stem cell
-Leading to increased production of RBCs, WBCs, and platelets.  patient has
↑blood viscosity and blood volume and congestion of organs and tissues with blood.
Pts. have hypercoagulopathies.
-Insidiously develops, and chronic, vacillating course.
-Median age diagnosis is 60 years, slight male predominance
-Some suggested that the disease associates with environmental exposure, but no
strong evidence.

2. Secondary polycythemia
a. Hypoxia driven – hypoxia stimulates erythropoietin(EPO) production in kidneys 
stimulates erythrocyte production
-May be due to high altitude, pulmonary disease, cardiovascular disease,
alveolar hypoventilation, defective O2 transport, or tissue hypoxia.
-Once Hb is stabilized at a higher level, EPO levels may return to normal.
-Physiologic response in which body tries to compensate for a problem, rather
than a pathologic response
b. Hypoxia independent
 -EPO is produced by malignant of benigh tumor tissues. EPO levels often
remain elevated.

Manifestations
Polycythemia vera
 First symptoms are circulatory manifestations because of the HTN caused by
hypervolemia and hyperviscosity. Symptoms include HA, vertigo, dizziness, tinnitus, visual
disturbances, generalized pruritus (exacerbated by hot bath. Related to histamine release
from an increased number of basophils), paresthesias and erythromelalgia (painful burning
and redness of hands and feet), angina, HF, intermittent claudication, and thrombophlebitis
(may be complicated by embolization. MOST common SERIOUS acute complications is
STROKE SECONDARY TO THROMBOSIS

 These manifestations are caused by blood vessel distention, impaired blood flow, circulatory
stasis, thrombosis, and tissue hypoxia caused by hypervolemia and hyperviscosity.

 Hemorrhagic phenomena may result in petechiae, ecchymoses, epistaxis or GI bleeding due

to vessel rupture from overdistention or inadequate platelet function. Hemorrhage can be
acute and catastrophic

 Hepatomegaly and splenomegaly – patient may complain of satiety and fullness

 Pain from peptic ulcer – due to increased gastric secretions or liver and spleen engorgement

 Plethora (ruddy complexion) – congestion causing distention of the blood vessels

 Hyperurecemia – uric acid is one of the products of cell destruction. If RBC destruction
increases, uric acid production also increases. May cause GOUT

Diagnostic Studies
Polycythemia vera
1) Elevated HB and RBC count with microcytosis
2) Low to normal EPO level (secondary polycythemia will have high level)
3) Elevated WBC with basophilia
4) Elevated platelets (thrombocytosis) and platelet dysfunction
5) Elevated leukocyte alkaline phosphatase, uric acid, and cobalamin levels
6) Elevated histamine levels
Bone marrow exam in polycythemia – shows hypercellularity of RBCs, WBCs, and platelets
Splenomegaly found in 90% with polycythemia vera but not secondary polycythemia.

Hb/H RB WBC platelets Uric Leukocyte histami cobalam EPO

ct C aci alkaline ne in
d phosphatase
Polycythe ↑ ↑ ↑ with ↑ ↑ ↑ ↑ ↑ Low to
mia vera basophil Thrombocyt normal
ia osis
And platelet
dysfunction
*secondary polycythemia will have high levels
of EPO

Collaborative Care
TREATMENT is directoward
1) Reducing blood volume and viscosity and
2) Bone marrow activity

Phlebotomy - MAINSTAY of TREATMENT

 Surgical opening of a vein to withdraw blood
To reduce hematocrit and keep it less than 45% to 48%.
300 to 500ml of blood removed starting from the time of diagnosis to every other day
UNTIL HCT REDUCE TO NORMAL LEVELS. Repeated phlebotomies  iron deficient,
although asymptomatic, AVOID IRON SUPPLEMENT
Hydration therapy – to reduce blood viscosity

To inhibit bone marrow activity, myelosuppressive agents such as busulfam (Myleran),

hydroxyurea (Hydrea), melphalan (Alkeran), and radioactive phosphorus may be given.

Nursing Management for Polycythemia Vera

 Polycythemia vera is not preventable. Requires ongoing evaluation

o Acute exacerbations of polycythemia vera –

-Nurse may either assist with or perform the phlebotomy – depending on hospital policies.
Phlebotomy may be needed q 2 to 3 months, reducing blood volume by about 500ml each
time. Evaluate for development of complications
-Hydration therapy – evaluate fluid intake and output to avoid fluid overload and
underhydration
-If myelosuppressive agents are used – administer drug as ordered, observe patient, and
teach patient about side effects.
-Nutritional status in collaboration with dietitian may be necessary – offset the inadequate
food intake that results from GI symptoms of fullness, pain, and dyspepsia
-Activities – to decrease thrombus formation –initiate active or passive leg exercise and
ambulation when possible
-Myelofibrosis and leukemia may develop (although low incidence) – may be caused by
chemotherapeutic drugs used to treat the disease or secondary to a disorder in the stem
cells that progresses to erythroleukemia

 Secondary polycythemia - Maintaining adequate oxygenation may prevent problems b/c

it is generated by any source of hypoxia
TEACH – stop smoking, avoid high altitudes, control chronic pulmonary disease

 Thrombosis (ex; stroke) is the major cause of morbidity and mortality

 Lymphomas
 Lymphomas are malignant neoplasms (cancer)
 Originates in the bone marrow and lymphatic structures resulting in the proliferation
(rapid reproduction) of lymphocytes.
 Two major types of lymphoma – Hodgkin’s lymphoma and non-Hodgkin’s lymphoma (NHL)

Hodgkin’s Lymphoma

 Hodgkin’s lymphoma (=Hodgkin’s disease)

 Malignant condition
 Characteristics : proliferation of Reed-Sternberg cells which are giant, abnormal
multinucleated cells located in the lymph nodes.
 Bimodal age-specific incidence (occurs most frequently from 15 to 35 yrs of age and above 50
yrs old)
 Prevalent in men

Etiology
 Cause remains unknown
 Several key factors play a role:
o Infection with Epstein-Barr virus (EBV)
o Genetic predisposition
o Exposure to occupational toxins
o Human immunodeficiency virus(HIV) infected patients have higher incidence

Pathophysiology
 In Hodgkin’s lymphoma, hyperplasia (excessive proliferation or reproduction) of monocytes
and macrophages destroys the normal structure of lymph nodes.

 MAIN diagnostics
-presence of Reed-Sternberg cells in the lymph node biopsy specimens

 Disease arise in a single location (mostly lymph nodes) and then spreads along adjacent
lymphatics. Cervical lymph nodes are first to be affected in most patients. If recurrent
disease, more diffuse (spread, scattered), not necessarily contiguous.  eventually infiltrates
other organs, especially LUNGS, SPLEEN, AND LIVER
-If above the diaphragm, it remains confined to lymph nodes for period of time.
-If below diaphragm, frequently spreads to extralymphoid sites such as liver.

Clinical Manifestations
 Onset of symptoms is insidious

 Initial development is lymphadenopathy (enlargement of nodes): separate nodes that

remain movable and nontender.
o Most common location is cervical, axilary, or inguinal lymphadenopathy
o Second most common is mediastinal node mass – may have cough, dyspnea, stridor,
and dysphagia

 General Manifestations: Painless unless they exert pressure on adjacent nerves OR

alcohol induced pain (cause is unknown), weight loss, fatige, weakness, fever, chills,
tachycardia, night sweats, generalized pruritus without skin lesions
o Those who have B symptoms has worse prognosis. B symptoms include: fever, night
sweats, and weight loss
 o Hepatomegaly and splenomegaly, and anemia are seen in more advanced disease.

 Other physical signs depends on disease location:

o intrathoracic involvement – superior vena cava syndrome
o Enlarged retroperitoneal nodes – palpable abdominal masses or interfere with renal
function
o Liver involvement – jaundice
o Extradural involvement – paraplegia due to spinal cord compression
o Bone involvement – bone pain

Diagnostic Studies and Staging Studies

a) Peripheral blood analysis:
o shows microcytic hypochromic anemia, neutrophilic leukocytosis (15,000 to 28,000/µl),
lymphopenia, and ↑platelet count, hypoferremia (excessive iron uptake by liver and
spleen), leukopenia, thrombocytopenia, superimposed hypersplenism(splenomegaly
with cytopenias), elevated leukocyte alkaline phosphate(from liver and bone
involvement), hypercalcemia (bone involvement), hypoalbuminemia (liver involvement)
b) Excisional lymph node biopsy:
o examine for presence of Reed-Sternberg cells and to identify the subtype (most
common is nodular sclerosing)
c) Bone marrow biopsy:
o important aspect of staging
o Reed-Sternberg cells may also be found in the bone marrow
d) Radiologic evaluation: Define all sites and determine the clinical stage of the disease.
o CT or MRI – intial staging tools
o PET (positron emission tomography with or without CT scans - used to assess the
response to therapy and to differentiate residual tumor from fibrotic masses after
treatment. These scans show mediastinal lymphadenopathy, renal displacement
caused by retroperitoneal node enlargement, abdominal lymph node enlargement, and
liver, spleen, bone, and brain infiltration.

Nursing and Collaborative Management

 Use diagnostic studies to stage the disease. Final staging is based on extent of the
disease(clinical stage), as well as the presence of B symptoms.
 Nomenclature used in staging involves
o Classification of A or B
- A = no constitutional symptoms
- B = fever, drenching sweats & 10% wt loss
o depending on whether symptoms are present when disease is found
o Roman numeral (I to IV) that reflects location and extent of the disease
o Elevated sedimentation rate
o Age ≥50 yrs
o Presence of a large mediastinal mass and low serum albumin, hemoglobin, and
lymphocyte counts may move an early stage (I or II) to an unfavorable prognosis,
needs more aggressive therapy.
 Tx- begins w accurate classification & staging
 Chemotherapy
o ABVD: Adriamycin, Vinblastine, Bleomycin , Dacarbazine. – STANDARD
-Those in early stage will receive 2 to 4 cycles of chemotherapy.
-Those with early-stage but unfavorable prognostic features (ex; presence of B
symptoms) or intermediate-stage disease will receive 4 to 6 cycles of chemotherapy
-Advanced stage: more aggressive treatment using 6 to 8 cycles
o MOPP (alternating with ABVD): Mechlorethamine, Oncovin, Procarbazine, and
Prednisone.
 o Stanford V and BEACOPP and ICE : more aggressive regimens.
 Radiation therapy
-Depends on sites of disease and presence of resistant disease after chemotherapy.

 TREATMENT OF CHOICE for advanced, refractory or relapsed Hodgkin’s lymphoma (stages

IIIB and IV) :
Intensive chemotherapy with or without autologous or allogeneic HSCT “AND”
Hematopoietic growth factors
(*HSCT allow pts to receive higher, potentially curative doses of chemotherapy while reducing life-
threatening leukopenia)
 The development of secondary malignancies such as acute myelogenous leukemia, non-
Hodgkin’s lymphoma, and solid tumors are serious consequences of the treatment for
Hodgkin’s lymphoma. Endocrine, cardiac, and pulmonary dysfunction may develop due to
long-term toxicities from treatment.

 Nursing Care
-Based on managing problems related to the disease (pain due to tumor), pancytopenia, and
other side effects of therapy. Supporting patient is important. Physical, psychosocial, and
spiritual consequences of patient’s disease must be addressed.
-TEACH! Fertility issues soon after diagnosis b/c this disease is frequently seen in adolescents
and young adults.

 Staging: HD is divided into stages according to the microscopic appearance of involved LN,
the extent & severity & prognosis

Stage I -Involvement of a single lymph node or a single extranodal site (spleen, thymus)

Stage II -Involvement of 2 or more lympn node on same side of diaphragm OR localized

involvement of an extranodal site and one or more lympn node regions of the same
side of the diaphragm. The # of anatomic sites are indicated by a subscript (e.g. IIз
)

Stage III -Involvement of Lymph nodes on both sides of the diaphragm. May include a single
extranodal site, the spleen, or both.
-Now subdivided into lymphatic involvement
-Stage III1: upper abdomen in the spleen (splenic, celiac, and portal
nodes)
-Stage III2: lower abdominal nodes in the periaortic, mesenteric, and
iliac regions

Stage IV -Diffuse or disseminated disease of one or more extralymphatic organs or tissues

with or without associated lymph node involvement.
-Extranodal site is identified as H, hepatic; L, Lung; P, pleura; M, marrow; D, dermal;
O, osseous

Non-Hodgkin’s Lymphoma
 Most commonly occurring hematologic cancer, fifth leading cause of cancer death.
 Affects all ages
 Heterogenous group of malignant neoplasms of primarily B-cell or T-cell origin which B-cell
lymphomas constitute about 90%.
 Variety of clinical presentations; may be slow or rapid developing disease

Etiology and Pathophysiology

 Cause is unknown
 Risk factors include:
o More common in those who have immunosuppressive medications (ex; to prevent
rejection following an organ transplant or treat autoimmune disorders)
o Those who received chemotherapy or radiation therapy
o Occupational exposure to carcinogens

 No hallmark feature in NHL. However, all NHLs involve lymphocytes arrested in various
stages of development.
Examples:
o lymphoblastic lymphoma and lymphoblastic leukemia(majority of dz within bone
marrow: result from malignant proliferation of small naïve B lymphocytes.
o Diffuse large B-cell lymphoma (most common in adults): neoplasm that originates
in the lymph nodes
o Burkitt’s lymphoma: highly aggressive, thought to originate from B-cell blast cells in
the lymph nodes.
o Follicular lymphoma: most common indolent(inactive) or low-grade lymphoma,
malignant group of B cells that occupy center of the lymph node as they approach the
end of maturation.

Clinical Manifestations
 Can originate outside the lymph nodes
 Unpredictable spreading
 Painless lymph node enlargement
 Majority have already disseminated disease at the time of diagnosis. Therefore, other
symptoms will be present depending the spread. (hepatomegaly with liver involvement,
neurologic symptoms with CNS disease)
 Other manifestations: airway obstruction, hyperuricemia, renal failure from tumor lysis
syndrome, pericardial tamponade, and GI complaints.
 High-grade lymphomas may have lymphadeopathy and B symptoms such as fever, night
sweats, and weight loss.
 Usually normal peripheral blood but some lymphomas manifest in “leukemic” phase.

Diagnostic and Staging

 Similar to Hodgkin’s lymphoma but more studies may be done due to extranodal sites
 MRI: to rule out CNS or bone marrow infiltration
 Barium enema or CT: to visualize suspected GI involvement
 Lymph node biopsy: establishes the cell type and pattern

 Establishing the precise histologic subtype is extremely important – use lymph node biopsy
the establish cell types and pattern.

 NHL is classified based on morphologic, genetic, immunophenotypic, and clinical features.

International Working Formulation (IWF) & Nursing and Collaborative

Management for Non-Hodgkin’s Lymphoma

Low-grade Small lymphocytic, - Median overall survival of 9 yrs

(Indolent) plasmacytoid - Relapse several times
lymphomas Follicular, predominantly - Cure is very unlikely
*Difficult to small cleaved cell - Observation until dz progression for asymptomatic
effectively treat. Follicular, mixed small patients with low-volume tumors and normal blood
cleaved and large cell counts.
- External beam irradiation for local, limited disease.
 - Therapy indicated when local symptoms from
progressive, bulky, or painful disease or
compromise of normal organ function
- OPTIONS:
- *Rituximab q weekly and then every 2 months as
maintenance therapy
Once disease is symptomatic, rituximab with
chemotherapy such as cyclophosphamide (Cytoxan)
with or without prednisone or even the CHOP
regimen may be used.
- Complete remissions are uncommon
- Majority responds with improvement in
adenopathy(swelling in lymph nodes) and
symptoms.
Intermediate- Follicular, predominantly
grade large cell
(Aggressive) Diffuse, small cleaved cell
lymphomas Diffuse, mixed, small and
large cell
Diffuse, large cell; cleaved
cell
Peripheral T cell
High-grade Large cell immunoblastic - Combination chemotherapy (high dose),
(Very Lymphoblastic aggressive with Rituximab and with localized
aggressive) Small noncleaved cell; radiation if needed
Lymphomas Burkitt’s -Most common standard chemotherapeutic regimen
*more aggressive is CHOP-R
the lymphoma, -RICE, EPOCH, CHAP, ESHAP
the more -Dose dense(intensified) treatment with CHOP-14 q
responsive to 2 weeks OR q 3 to 4 weeks with hematopoietic
treatment and growth factor
curing. -High-dose chemotherapy with autologous HSCT –
better outcome

Some subtypes may be treated differently than general standards b/c NHL represents a large
variety of neoplasms
Cutaneous T-cell - Topical corticosteroids or topical chemotherapy for limited-stage disease
lymphoma
More diffuse - Phototherapy, α-interferon, oral bexarotene (Targretin), a retinoid, or denileukin
disease diftitox (Ontak), which is a novel fusion protein consisting of interleukin-2 and
diphtheria toxin
Other therapies
Primary used for - Monoclonal antibodies ibritumomab tiuxetan (Zevalin) and
patients with tositumomab (Bexxar). – these antibodies targets the CD20 antigen which is on
indolent the surface of mature B cells and B-cell tumors. These antibodies are linked to
lymphomas, radioactive isotope so it allows for the delivery of radiation directly to the
particularly those malignant cells.
with - SIDE EFFECTS: Pantocytopenia
chemotherapy- - Minimize rhe risk of radiation exposure to staff and others
refractory
disease.

 Factors considered with NHLs: advanced disease, number of extranodal sites, older than 60
yrs of age, high serum lactate dehydrogenase, and performance status.

 For making therapeutic decisions and assessing prognosis: immunologic, cytogenetic, and
molecular studies are useful

 Hodgkin’s lymphoma has better prognosis than NHLs

Nursing and Collaborative Management for Non-Hodgkin’s Lymphoma

*Rituximab –
- used to treat NHL
-Genetically engineered monoclonal antibody against the CD20 antigen on the surface of
normal and malignant B lymphocytes. Once bound to cell, causes lysis and cell death.

Nursing Care
 Similar to Hodgkin’s lymphoma
 Manage problems related to the disease (ex; pain due to tumor, spinal cord compression,
tumor lysis syndrome), pancytopenia, and other side effects of therapy.
 Important for the nurse to have an understanding of the subtype and extent of the disease
b/c NHL are more extensive and involves specific organs (ex; CNS, spleen, liver, GI tract, bone
marrow)
Hannah’s bonus question #1
Q: patient with NHL involving the colon shows symptoms such as abdominal
guarding and enlarged and tympanic abdomen. As a nurse, what do you think is
happening to the patient ?
A: could indicate bowel perforation and considered a medical emergency.
 Patient with Burkiitt’s NHL starting chemotherapy – high risk for tumor lysis syndrome, have
frequent lab drawn and monitored, strict intake and output
 Care for radiation therapy: skin and safety issues
 Psychosocial considerations are important
 Fertility issues
 Long term evaluation is important

Multiple myeloma

 Multiple myeloma, or plasma cell myeloma, is a condition in which neoplastic plasma cells
infiltrate the bone marrow and destroy bone.
 More common in men, African Americans, usually develops after 40 years of age
 Lives approx 2 years after diagnosis IF untreated.
 Myeloma cells tend to collect in the bone marrow and in the hard, outer part of bones.
Sometimes they collect in only one bone and form a single mass, or tumor, called a
plasmacytoma. In most cases, however, the myeloma cells collect in many bones, often
forming many tumors and causing other problems. When this happens, the disease is called
multiple myeloma.

Etiology
 Cause unknown
 Genetic factors, viral infection, exposure to radiation, organic chemicals (ex;benzene),
herbicides, and insecticides may play a role of developing multiple myeloma.

Pathophysiology
 Neoplastic plasma cells (activated B cells) infiltrate bone marrow, produce abnormal and
excessive amounts of immunoglobulin (myeloma protein or M protein)(usually IgG –most
common, IgA,IgD, or IgE) and cytokines (interleukins ; IL-4, IL-5, and IL-6) – plays an
important role in bone destruction.

 Normal plasma cells are reduced

 -Result in end-organ effects of myeloma, destroying the bone, and bone marrow, nodes,
lymph, liver, spleen, kidneys, and heart muscles are invaded

 Bence Jones proteins (free light-chain proteins) from the myeloma cell can be detected in
urine in some patients.

Manifestations
 Multiple myeloma develops slowly and insidiously. The patient often does not manifest
symptoms until the disease is advanced.
 SKELETAL PAIN (major symptom) – pain in the pelvis, spine, and ribs is common and it’s
triggered by movement.
 Diffuse osteoporosis develops, osteolytic lesions are seen in the skull, vertebrae, and ribs.
 Vertebral destruction, possible paraplegia, and fractures due to loss of bone integrity
 Hypercalcemia; renal, GI, or neurologic manifestations such as polyuria, anorexia, confusion,
and seizures, coma and cardiac problems
 High protein levels(myeloma protein); renal tubular obstruction, interstitial nephritis, renal
failure
 Anemia, thrombocytopenia, neutropenia, granulocytopenia

Diagnostic Studies
 Monoclonal (M) an antibody protein can be found in blood and urine
 Pancytopenia, hypercalcemia, presence of Bence Jones protein in the urine, and elevated
serum creatinine
 x-rays – show bone erosions, generalized thinning of bones, fractures, vertebrae, ribs, pelvis,
and bones of thigh and upper arm.
 Bone marrow analysis – shows increased numbers of plasma cells
 TWO MARKERS to measure prognosis
 β2-microglobulin and albumin (higher levels of β2-microglobulin and lower levels of
albuminpoorer prognosis)

Collaborative Care
 GOAL - Manage both the disease and its symptom, control pain, prevent fractures.

 Multiple myeloma is seldom cured, but treatment can relieve symptoms, produce remission,
and prolong life.
Current treatment options – watchful waiting (for early multiple myeloma), corticosteroids,
chemotherapy, biologic therapy, and hematopoietic stem cell transplantation (HSCT)
 Ambulation, adequate hydration – treats hypercalcemia, dehydration, and potential renal
damage

 Analgesics, orthopedic support, localized radiation therapy help reduce skeletal pain
Biphosphonates such as pamidronate (Aredia), zoledronic acid (Zometa), and etidronate
(Didronel) – give monthly by IV infusion. inhibits bone breakdown and used from skeletal pain
and hypercalcemia

 Vertebroplasty to support degenerative vertebrae - surgery

 CHEMOTHERAPY - first treatment recommended for multiple myeloma – to reduce number

of plasma cells
Alkylating drugs includes melphalan (Alkeran), cyclophosphamide (Cytoxan), vincristine
(Oncovin), and doxorubicin (Adriamycin).
Corticosteroids (prednisone, dexamethasone[Decadron]) may be added – they show
antitumor effect in some patients.
 COMMON REGIMEN is VAD – vincristine, doxorubicin (Adriamycin), and dexamethason
---give once a month over several months before an autologous HSCT. After autologous
HSCT, high-dose chemotherapy such as melphalan evolved as the standard of care

 Other meds to keep myeloma under control

Bortezomib (Velcade) – new, proteasome inhibitor.
Proteasomes are present in all cells and regulate cell growth. (Normal cells recover from
proteasome inhibition, but Cancer cells die when inhibited).
Indicated for patients whos disease relapsed after two prior treatments and who have
demonstrated resistance to their last treatment.
Thalidomide (Thalomid)
Immune-modulating and antiangiogenic drug – slow the growth of plasma cells and reduce
their numbers
Give alone or with corticosteroids or chemotherapy drugs
Contraindicated in pregnant women
Associates in DVT, neuropathies, and excess somnolence
α-interferon therapy following chemotherapy - altersthe receptors for IL-6 (growth factor for
myeloma cells)

 TREATMENT FOR COMPLICATIONS

Allopurinol (Zyloprim) – reduce hyperuricemia
IV furosemide (Lasix) – promote renal excretion of calcium
Calcitonin and pamidronate –treats hypercalcemia and decrease risk of fractures and reduce
bone pain

Nursing management for Multiple Myeloma

 Maintain adequate hydration:3-4L/d for output of 1.5-2L/d. MONITOR electrolyte and fluid
balance for renal dysfunction
 Assist with ambulation: weight bearing to reabsorb calcium
 Adm. Corticosteroids to help excrete calcium; allopurinol (antigout-xanthine oxidase
inhibitors)for hyperuremia
 Pain management with analgesics (NSAIDs, acetaminophen, or acetaminophen/opioid
combination) better than opioid alone in diminishing bone pain, braces, local radiation
 Prompt treatment of Infection
 nurse must be careful when moving and ambulating the patient – due to potential for
pathologic fractures.
 Peripheral neuropathy is common – prevent falls.
 Psycosocial: support adaptation to chronic nature- symptoms remit and exacerbate,
ultimately disease becomes resistant to treatment
 See anemia (NCP 31-1), thrombocytonemia (NCP 31-2), neutropenia (NCP 31-3)

Disorders of the Spleen

 Located in the upper left quadrant next to kidney
 Functions can be classified as
– Hematopoietic: Able to produce RBCs during fetal development
– Filtration
 Remove old and damaged RBCs from circulation
 Removes hemoglobin from RBCs and returns iron component to the bone
marrow for reuse
 Filters out bacteria, especially encapsulated organisms
 Normal spleen contains 20 to 40 ml of blood. Do not usually serve as reservoir for blood
volume and erythrocytes.

 Spleen can be affected by many illness may cause splenomegaly (enlarged spleen).
Degree of enlargement varies with dz. Ex) Chronic myelogenous leukemia, hairy cell
leukemia, and thalassemia major causes massive splenic enlargement. Heart failure and SLE
causes mild splenic enlargement.

 Hypersplenism – occurrence of splenomegaly and peripheral cytopenias (anemia,

leukemia, and thrombocytopenia)

Manifestations
 When spleen enlarges, normal filtering and sequestering capacity increases.  reduction in
number of circulating blood cells
 Howell –Jolly bodies or pitted or pocked erythrocytes – unusual findings in the peripheral
smear
 Slight to moderate enlargement : usually asymptomatic, may be found during routine
examination of abdomen.
 Massive enlargement : some tolerate, but complain of abdominal discomfort and early
satiety.

Techniques to assess size of spleen (diagnostic tests)

 Physical examination, Tc-sulfer colloid liver-spleen scan, CT scan, MRI, and ultrasound scan

Treatment of splenomegaly
 Laparoscopy or open laparotomy and splenectomy( has dramatic effect in increasing
peripheral RBC, WBC, platelet)
Splenectomy may be done due to splenomegaly, splenic rupture (from trauma, during other
surgical procedures, disease such as mononucleosis, malaria, and lymphoid, neoplasms)

Nursing Management
 Pain management : may require analgesics
 May impair diaphragmatic excursion : evaluate lung expansion
 care in moving, turning and positioning
 prevent life-threatening complications if hypersplenism
 post op splenectomy : special observation for hemorrhage which can lead to shock,
fever, and abdominal distention. Immunologic deficiencies may develop. (↓IgM levels,
normal IgG and IgA). May have lifelong risk for infection (especially pneumococcus) – TEACH
for pneumovax immunization

Blood Component Therapy

 Blood component therapy is frequently used in managing hematologic diseases. However,

blood component therapy only temporarily supports the patient until the underlying problem
is resolved.
 Use only if necessary
 Avoid developing a complacent attitude about this common but potentially dangerous
therapy
 Make sure PHYSICIAN discuss risks, benefits, and alternatives with the patient and that this is
documented.
 Blood Transfusion has a broader meaning today. Not just whole blood (used rarely for
massive hemorrhage) but also specific components of blood such as platelets, packed red
blood cells (PRBCs), or plasma

Administration Procedure
 If using small guage needles : split unit (the blood bank issues half of the unit at a time) or
dilute with NS to ensure that it does not run over the MAXIMUM TIME OF 4 HOURS due to
bacterial growth. Blood unrefrigerated for 4 hoursreturn to blood bank.
o 23-guage needle: safe, but usually impedes flow, cause hemolysis particularly when
using pressured infusion device. Smaller needles can be used for platelets, albumin,
and clotting factor replacement.
o 19-guage needle preferred running into a free-flowing line.
o 16 to 18 guage needle : for rapid transfusions
 “Y-type” tubing with a microaggregate filter (filters out particulate) with one Y for the isotonic
saline solution and other Y for the blood product.
 DO NOT USE Dextrose Solutions or Lactated Ringers : they induce RBC hemolysis
No other additives (inducing medications) should be given via same tubing as blood UNLESS
tubing is cleared with saline solution

 When the blood or blood components have been obtained from the blood bank, POSITIVE
IDENTIFICATION OF THE DONOR BLOOD AND RECIPIENT MUST BE MADE. Improper
product-to-patient identification causes 90% of hemolytic transfusion reactions.

 The blood bank is responsible for typing and crossmatching the donor’s blood with
recipient’s blood : result of testing should be noted on the product bag or tag.

Nursing Management
 Make sure pt agrees and understands the procedure, and signs and symptoms to report.
 Take V/S for baseline: If ANY abnormals, report to MD to clarify when to administer blood.
 Administer as SOON AS it is brought to patient: DO NOT refrigerate in nursing unit. IF NOT
USED WITHIN 30 MINUTES, RETURN TO BLOOD BANK!!
 DURING FIRST 15 MINUTES or 50ML OF INFUSION- STAY WITH THE PT b/c untoward
reactions most likely to occur at this time. Infusion rate should be no more than 2ml/min
during thise period.
 PRBCs should not be infused quickly UNLESS EMERGENCY b/c may cause chills due to cold
product. IF rapid replacement is necessary, blood-warming device may be used. Fresh frozen
plasma and platelets, may be infused over 15 to 30 minutes.
 After 15 minutes – retake V/S. rate of infusion is governed by clinical condition of the
patient AND the product being infused. If not danger of fluid overload, most patients can
tolerate of 1 unit of PRBCs over 2 hours.

Blood Transfusion Reactions

 A blood transfusion reaction is an adverse reaction to blood transfusion therapy that
can range in severity from mild symptoms to a life-threatening condition. Blood
transfusion reactions can be classified as acute or delayed.
If acute transfusion raction occurs …
1) Stop the transfusion
2) Maintain a patent IV line with saline solution
3) Notify blood bank and MD immediately
4) Recheck identifying tags and numbers
5) Monitor vital signs and urine output
6) Treat symptoms per physician order
7) Save the blood bag and tubing and send them to blood bank for examination
 8) Complete transfusion reaction reports
9) Collect required blood and urine specimens at intervals stipulated by hospital policy to
evaluate for hemolysis
10) Document on transfusion reaction form and patient chart.

 Blood bank and Labs are responsible for identifying the type of reaction

Acute Transfusion Reactions

Reaction Cause Manifestatiosn Management Prevention

Acute - infusion of ABO- - Chills, fever, low - Treat shock and DIC Meticulously
Hemolytic incompatible back pain, if present verify and
components or flushing, - Draw blood samples document pt’s
components tachycardia, for serologic testing identification
containing 10 dyspnea, slowly to avoid from sample
mL or more of tachypnea, hemolysis from collection to
RBCs. hypotension, procedures. Send component
- Antibodies in vascular urine specimen to lab infusion.
recipient’s collapse, - Maintain BP with IV
plasma attach hemoglobinuria, colloid solutions.
to antigens on acute jaundice, Give diuretics as
transfused red dark urine, prescribed to
blood cells, bleeding, acute maintain output.
causing renal failure, - Insert urinary
agglutination of shock, cardiac catheter or measure
cells(blocks arrest, death. voided amounts to
blood flow) and - Acute : first 15 monitor urine output
RBC destruction min of - Dialysis may be
- Mislabeling transfusion required if renal
specimens - Delayed: 2 to 14 failure occurs
- Adm. To wrong days after - Do not transfuse
pt. transfusion additional RBC
containing
components until
blood bank has
provided newly
crossmatched units
Febrile - Sensitization to - Sudden chills - Give antipyretics as consider
(nonhemoly donor WBCs and fever >1C prescribed-AVOID leukocyte-poor
tic) (leukocyte rise, HA, ASPIRIN in blood products
Reactions incompatibility- flushing, thrombocytopenic (filtered, washed,
most common) anxiety, patients or frozen) for
MOST platelets, or vomiting, - DO NOT RESTART patients with a
COMMON plasma proteins. muscle pain TRANSFUSION unless history of two or
physician orders more such
reactions.
Mild allergic - Sensitivity to - Flushing, - Give antihistamine, -Treat
reactions foreign plasma itching, corticosteroid as prophylactically
proteins uticaria(hives) directed with
- More common - If symptoms are mild antihistamines
in individuals and transient, -Consider washed
with Hx of transfusion may be RBCs and
allergies restarted slowly platelets.
- Do not restart
transfusion if FEVER
or pulmonary
symptoms develop
Anaphylacti - Sensitivity to - Anxiety, - Initiate CPR, if -Transfuse
c and donor plasma urticaria, indicated extensively
severe proteins dyspnea, - Have EPINEPHRINE washed RBC
allergic - Infusion of IgA wheezing, ready for injection products, from
reactions proteins to IgA- progressint to - Do NOT RESTART which all plasma
deficient dyanosis, TRANSFUSION has been
recipient who bronchospasm, removed.
has developed hypotension, -Use blood from
IgA antibody shock, and IgA-deficient
possible cardiac donor
arrest. -Use autologous
components
Circulatory - Fluid - Cough, - Place pt upright with -Adjust
overload administered dyspnea, feet in dependent transfusion
faster than the pulmonary position volume and flow
circulation can congestion, HA, - Adm. Prescribed rate based on pt.
accommodate HTN, diuretics, oxygen, size and clinical
- Individual tachycardia, JVD morphine status
w/cardiac or - Complain of - Phlebotomy may be -Have blood bank
renal SOB and indicated divide unti into
insufficiency are adventitious smaller aliquots
at risk breath sounds for better spacing
- Elderly at risk of fluid input

Sepsis Transfusion of - Rapid onset of - Obtain culture of pt’s -collect, process,

bacterially infected chills, high blood and send bag store, and
blood fever, vomiting, with remaining blood transfuse blood
diarrhea, and tubing to blood products
marked bank for further study according to
hypotension, or - Treat septicemia as blood banking
shock directed – antibiotics, standards and
IV fluids, infuse within 4 hr
vasopressors of starting time.

Transfusion - Reaction btwn - Fever, - Send bag with Provide leukocyte

related transfused hypotension, remaining blood and reduced
acute lung antileukocyte tachypnea, tubing to blood bank products.
injury antibodies and dyspnea, for further study; Identify donors
(TRALI) recipient’s decreased O2 draw blood for who are
leukocytes, sat, frothy arterial blood gases implicated in
causing sputum and HLA or TRALI reactions
pulmonary - Non-cardiogenic antileukocyte and DO NOT
inflammation pulmonary antibodies; obtain ALLOW THEM TO
and capillary edema (acute chest x-ray DONATE
leak lung injury) - Provide oxygen and
administer
corticosteroids
(diuretics of no
value?)
- Initiate CPR if needed
and provide
ventilator and blood
pressure support if
needed
- - -

Massive Blood Transfusion Reaction

• An acute complication of transfusing large volumes of blood products is termed massive
blood transfusion reaction. Massive blood transfusion reactions can occur when
replacement of RBCs or blood exceeds the total blood volume within 24 hours.
Delayed Transfusion Reactions

 Delayed transfusion reactions include delayed hemolytic reactions, infections, iron overload,
and graft-versus-host disease.
 Infectious agents transmitted by blood transfusion include hepatitis B and C viruses, HIV,
human herpesvirus type 6, Epstein-Barr virus, human T cell leukemia, cytomegalovirus, and
malaria.

AutoTransfusion
 Autotranfusion, or autologous transfusion, consists of removing whole blood from a
person and transfusing that blood back into the same person. The problems of
incompatibility, allergic reactions, and transmission of disease can be avoided.