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Indriani Silvia

Patologi Klinik
FK UNSWAGATI
06092015

Kelainan pada hematologi


Jenis kelainan hematologi
Gambaran kelainan hematologi
pada pemeriksaan laboratorium
Kelainan onkologi
Jenis pemeriksaan onkologi
Indikasi dan tujuan pemeriksaan
Interpretasi hasil pemeriksaan

Transports oxygen, called


oxyhemoglobin, when it gives up its
oxygen it is deoxyhemoglobin.

Also binds and transports carbon


dioxide, carbaminohemoglobin.

Makes up 97 % of RBC 250


million Hb molecules per RBC

Men: 14-18 mg/dl blood

Women: 12-16 mg/dl blood

Types of Hemoglobin (Hb)


Hb A
96% of adult Hb (2 2)

Hb A2
3% of adult Hb (2 2)

Hb F
1 % of adult Hb (2 2)

Liver contains phagocytic cells known

as Kupffer cells that act as a filter for


damaged or aged cells in a manner
similar to, but less efficient than the
phagocytic cells in the spleen.
If the bone marrow cannot keep up with the
physiologic demand for blood cells, the liver may
resume the production of blood cells that it
began during fetal life

Blood
group
A

Antigen

Antibody

Donor to

Anti-B

Recipient
from
A, O

Anti-A

B, O

AB

AB

Neither

AB

A, B, AB, O

Neither

AntiA/Anti-B

O, A, B, AB

Universal donor "O"


Universal recipient "AB"

Acute Hemolytic Reaction


Symptoms

Fever, chills and fever, the feeling of heat along

the vein in which the blood is being transfused


Pain in the lumbar region
Constricting pain in the chest, tachycardia,
hypo-tension
Hemoglobinemia with subsequent hemoglobinuria and hyperbilirubin-emia.
"feeling of impending doom"

Acute Hemolytic Reaction


Causes
Human error!
Transfused red cells react with circulating antibody in
the recipient with resultant intravascular hemolysis

Acute Hemolytic Reaction


Frequency
Rare

Prevention
Proper identification of patients, pretransfusion

blood samples and blood components at the


time of transfusion

Delayed Hemolytic Reaction


Falling hematocrit

due to extravascular destruction of the transfused

red blood cells)

Positive

(DAT)

Occurs

direct antiglobulin (Coombs) test

about 4-8 days after blood transfusion

Patients

may manifest fever and leukocytosis

Appearing to have an occult infection.

Febrile Transfusion Reaction


Fever or chill fever
temperature rise of 1.5 F or 1.0 C from the

baseline

Cytokines and antibodies to leukocyte


antigens reacting with leukocytes or
leukocyte fragments
1 in 8 transfusions

Allergic urticaria
Laryngeal edema and bronchospasm
1% of recipients
If coupled with another sign, such as fever,

evaluation for a hemolytic reaction may be


indicated.

Allergic Anaphylaxis
Anaphylactic or anaphylactoid
Respiratory involvement with dyspnea or stridor

Cardiovascular instability
hypotension, tachycardia, loss of

consciousness, cardiac arrhythmia, shock and


cardiac arrest

Volume Overload
Transfusion-related volume overload
Infuse smaller volumes more slowly

Bacterial Contamination
Hypotension, shock, fever and chills,
nausea and vomiting, and respiratory
distress
Gram stain and blood culture

Follow protocol for transfusion


reactions implemented by the
institution

Stop the transfusion immediately!

Disconnect the intravenous line from


the needle.

Seek medical attention immediately. If


the patient is suffering cardiopulmonary
collapse, and medical attention is not
immediately available, press for Code"

Check to ensure that the patient name


and registration number on the blood bag
label exactly with information on the
patient's identification

Do not discard the unit of blood that


has been discontinued because it may be
necessary for the investigation of the
transfusion reaction.

Reaction Type

Treatment - Adult

Pediatric

Follow-up

Acute
Hemolytic
Reactions

Diuretic therapy: Initially, give 40-80


mg Furosemide (Lasix)
intravenously. This dose can be
repeated once. Lack of response to
furosemide in 2-3 hours indicates
the presence of acute renal failure.

Pediatric dose: 1-2


mg/kg/dose.
May repeat once
at 2-4 mg/kg.

Treat shock and disseminated


intravascular coagulation with
appropriate measures if and when
they appear.

Water loading: The patient should be


hydrated to maintain urinary
output of at least 100 mL/hr until
urine is free of hemoglobin.
Infuse a loading dose of 0.9% sodium
chloride or 5% dextrose in 0.45%
sodium chloride. Chart hourly
urine output. Maintain the urine
output by administering
intravenous fluid at 100 mL/hour
until the urine is free of
hemoglobin. If the patient's
urinary output does not increase,
with this hydration any additional
fluids should be infused with
caution.

Pediatric patients
should receive a
smaller loading
volume of fluid
in proportion to
their body
surface area.

Reaction Type

Treatment - Adult

Delayed
Hemolytic
Transfusion
Reactions

Specific treatment generally is not


necessary

Pediatric

Follow-up

Supplemental transfusion of blood lacking


the antigen corresponding to the
offending antibody may be necessary
to compensate for the transfused cells
that have been removed from the
circulation.

Reaction Type

Treatment - Adult

Pediatric

Follow-up

Allergic
Transfusion
Reactions

Antihistamines(e.g., Benadryl). Give


50-100 mg orally or intravenously. If
urticaria develops slowly,
antihistamines may be given orally.

Pediatric dose: 1-2


mg/kg intramuscularly
or intravenously for
25-50 mg per average
dose.

Routine use of Benadryl as premedication


for all transfusions, regardless of a history
of allergic reactions, is discouraged.

Aminophylline for wheezing, at a dose


of 125-250 mg intravenously slowly
over a period of about five minutes

Pediatric dose: 3
mg/kg/dose in
intravenous drip over
of 20 minutes.

Epinephrine for severe, acute reactions


including laryngeal edema or
bronchospasm Give 0.1-0.5 mg (0.1-0.5
mL of a 1:1000 solution)
subcutaneously. Subcutaneous dose
may be repeated at 10-15 minute
intervals. The total subcutaneous dose
in a 24-hour period, with rare
exception, should not exceed 5 mg.

Pediatric dose: 0.03


mL/M2 (0.03 mg/M2
of a 1:1000 solution)
given subcutaneously.
A single pediatric
dose should not
exceed 0.3 mg.

Reaction Type

Treatment - Adult

Pediatric

Follow-up

Febrile
Transfusion
Reactions

Premedicate the patient with


acetaminophen or other
antipyretic agents when previous
reactions have been extremely
bothersome. Pediatric dose: 10
mg/kg to a maximum of 600 mg.

Aspirin will adversely affect the patient's


platelet function, so non-aspirin
antipyretic agents are preferable.

Severe shaking
Chills

(rigors) can be controlled by the


sedative effect of Benadryl or
Demerol (25-50 mg given
intramuscularly or intravenously

Note: Demerol may cause acute


respiratory arrest. An opiate
antagonist (Narcan) should be
immediately available.

Sepsis Due to
Bacterial
Contamination
of Donor Blood

Treatment of septic shock includes:


terminating the suspected
transfusion immediately, cardiovascular and respiratory support,
blood culture of the patient, and
administration of broad spectrum
antibiotics including antipseudomonas coverage if the
blood component involved is Red
Blood Cells.

Abnormally low number of RBC or Hb


levels
Reduced oxygen carrying capacity
Causes
Blood loss
Increased rate of red cell destruction

Hemolytic anemia

Deficient or impaired red cell production

Risk factors
Poor diet
Intestinal disorders
Menstruation
Pregnancy
Chronic conditions
Family history

NOT A DISEASE but a symptom


Dependent on severity, speed of development,

age, health status and compensatory


mechanisms
Associated with impaired O2 transport,
alteration in RBC structure or with chronic
illness
Not expressed until 50% of RBC mass is lost

Signs and symptoms


The main symptom of most types of
anemia is fatigue

Weakness
Pale skin
Tachycardia
Shortness of breath
Chest pain
Dizziness
Cognitive problems
Numbness or coldness in your extremities
Headache

Iron Deficiency Anemia


Most common form of anemia

Affects about one in five women


Half of pregnant women and 3 percent of men

in the United States.

The cause is a shortage of the element iron


Nutritional imbalance
Slow, chronic bleeding disorders
Inability to recycle plasma iron

Vitamin Deficiency Anemias


Folate and vitamin B-12 deficiency
Intestinal disorder that affects the
absorption of nutrients
Fall into a group of anemias called
megaloblastic anemias, in which the bone
marrow produces large, abnormal red
blood cells.

Anemia of Chronic Disease


Interfere with the production of red blood
cells, resulting in chronic anemia
Kidney failure also can be a cause of
anemia
The kidneys produce a hormone called

erythropoietin, which stimulates your bone


marrow to produce red blood cells.

A shortage of erythropoietin, which can result from


kidney failure or be a side effect of chemotherapy, can
result in a shortage of red blood cells.

Aplastic Anemia
Life-threatening anemia caused by a
decrease in the bone marrow's ability to
produce all three types of blood cells red
blood cells, white blood cells and platelets
Cause of aplastic anemia is unknown
autoimmune disease
Chemotherapy
Radiation therapy
Environmental toxins

Anemias associated with bone marrow


disease
Leukemia and myelodysplasia, can cause
anemia by affecting blood production in
the bone marrow
Effects vary from a mild alteration in blood
production to a complete, life-threatening
shutdown of the blood-making process
Myelodysplasia is a pre-leukemic
condition that can cause anemia.
Other cancers of the blood or bone marrow,
such as multiple myeloma, myeloproliferative
disorders or lymphoma, can cause anemia.

Hemolytic Anemias
Red blood cells are destroyed faster than
bone marrow can replace them.
Autoimmune disorders can produce
antibodies to red blood cells, destroying
them prematurely
Hemolytic anemias may cause yellowing of the

skin (jaundice) and an enlarged spleen.

Hereditary Spherocytosis
Mutations in the ankyrin molecule with a
secondary deficiency of spectrin along the
cell membrane
Reduced red cell stability
Does not affect oxygen carrying capacity
Splenic sequestration

Sickle cell anemia


Defective form of hemoglobin that forces
red blood cells to assume an abnormal
crescent (sickle) shape.

Mutation for the gene coding for the -globulin

chain

Valine is substituted for glutamic acid

HbS

Red cells die prematurely, resulting in a


chronic shortage of red blood cells.

Block blood flow through small blood vessels in

the body, producing other, often painful,


symptoms.

Single base pair mutation results in a


single amino acid change.
Under low oxygen, Hgb becomes
insoluble forming long polymers
This leads to membrane changes
(sickling) and vasoocclusion

Sickle Cell Mutation

Chromosome16
5'
Chromosome11
5'

Normal (HbA)
CCTGAGGAG

-Pro-Glu-Glu-

567
-O2
+O 2

3'
3'

Abnormal(HbS)
CCTGTGGAG

-Pro-Val -Glu-

567

-O 2
+O2

Deoxygenation of SS erythrocytes leads to


intracellular hemoglobin polymerization, loss of
deformability and changes in cell morphology.

OXY-STATE

DEOXY-STATE

- Thalassemia
Common in Asians
Deletion of globulin chain loci
4 possible degrees of thalassemia:
Silent carrier, loss of a single globulin gene
thalassemia trait, loss of a pair of globulin

gene
HbH disease, only a single gene is present
Hydrops fetalis, deletion of all globulin

The only treatments are stem cell


transplant and simple transfusion.
Chelation therapy to avoid iron
overload has to be started early.

TALASSEMIA

An acquired disorder of the bone marrow


that causes the overproduction of all three
blood cell lines
white blood cells, red blood cells, and platelets

It is a rare disease that occurs more


frequently in men than women, and rarely
in patients under 40 years old.
causes is unknown

Usually develops slowly, and most patients


are asymtomatic
abnormal bone marrow cells proliferate

uncontrollably leading to acute myelogenous


leukemia

Patients have an increased tendency to


form blood clots that can result in strokes
or heart attacks
Some patients may experience abnormal

bleeding because their platelets are abnormal

Symptoms

Headache
Dizziness
Pruritus
Fullness in the left upper abdomen
Erythema (face)
Shortness of breath
Orthopnea
Symptoms of phlebitis

Collectively known as White Blood Cells


(WBC)

Formed elements of the blood with


organelles and a nucleus but lack
hemoglobin

Protect the body against microorganisms


and remove dead cells and debris from the
body

Per l blood

Per l of blood
5,000 10,000

Total WBC count


Neutrophils

50 - 70%

2,000 7,000

Lymphocytes

20 - 40%

1,000 4,000

Monocytes

1 6%

50 600

Eosinophils

1 5%

50 500

Basophils

0 2%

0 - 100

Leukopenia
Decreased peripheral white cell count due to

decrease numbers of any specific types of


leukocytes

Leukocytosis
Nonneoplastic elevation of WBC count

Neutropenia
Reduction in the number of granulocytes
(<1500/l)
Increased risk of infection
Reduced phagocytosis response

Neutropenia
Decreased or defective granulopoiesis
Aplastic anemia
Anti-neoplastic agents
Other drugs: chloramphenicol, sulfonamides,

chlorpromazine

Accelerated removal or destruction


Aggressive and chronic infections

Manifestation of Neutropenia
Infections
Signs and Symptoms
Malaise, chills, fever
Ulcerative necrotizing lesions of the mouth,
skin vagina and GI tract

Reactive Leukocytosis
Increase number of WBC
Common reaction due to a variety of
inflammatory states caused by microbial or
non-microbial stimuli
Usually non-specific

Causes of Leukocytosis
Polymorphonuclear leukocytosis

Acute bacterial infections

Eosinophilic leukocytosis

Allergic disorders

Monocytosis

Chronic infections

Lymphocytosis

Chronic immunologic disease

Leukemoid reaction this is an extreme neutrophilia with a


WBC count > 30 x 109/L
Many bands, metamyelocytes, and myelocytes are seen
Occasional promyelocytes and myeloblasts may be seen.
This condition resembles a chronic myelocytic leukemia
(CML), but can be differentiated from CML based on the
fact that in leukemoid reactions:
There is no Philadelphia chromosome
The condition is transient
There is an increased leukocyte alkaline phosphatase score
(more on this later)
Leukemoid reactions may be seen in tuberculosis, chronic
infections, malignant tumors, etc.

1.
2.
3.

4.

Leukemia neoiplasms of the


hematopoietic stem cells
Malignant lymphomas cohesive tumor
lesions; neoplastic lymphocytes
Plasma cell dyscrasias arising from the
bones; localized disseminated
proliferation of antibody forming cells
Histocytoses proliferative lesions of
histiocytes

Leukemia
Malignant neoplasm of the hematopietic
stem cells
BM replaced by unregulated, proliferating,
immature neoplastic cells blood
leukemia enter spleen, lymph nodes
Most common cancer in the paediatric age
Leading cause of death in children
between 3 and 14 years old

Classification of Leukemia
A.
According to cell type and state of cell
maturity

Lymphocytic immature lymphocytes and


their progenators
Myelocytic pluripotent myeloid stem cells
and interferes with maturation of all
granulocytes, RBC and platelets

Acute or Chronic

B.

Acute immature cells (blast)


Chronic well differentiated leukocytes

Congenital
Pelger-Huet anomaly
Bilobed and occasional unsegmented neutrophils
Autosomal recessive disorder

Neutrophil hyper-segmentation
Rare autosomal dominant condition
Neutrophil function is essentially normal

May-Hegglin anomaly
Neutrophils contain basophilic inclusions of RNA
Occasionally there is associated leucopenia
Thrombocytopenia and giant platelet are frequent

Alders anomaly
Granulocytes, monocytes and lymphocytes contain granules
which stain purple with Romanowsky stain
Granules contain mucopolysaccharides

Chediak-Higashi syndrome
Autosomal recessive disorder
Giant granules in granulocytes, monocytes and
lymphocytes
Partial occulocutaneous albinism
Depressed migration and degranulation
Recurrent pyogenic infections
Lymphoproliferative syndrome may develop
Treatment is BMT

Acquired

Toxic granulation
Dohle bodies
Pelger cells
Hypersegmented neutrophils

Leucocyte adhesion deficiency


Chronic granulomatous disease
Chediak-Higashi syndrome
Primary immunodeficiency

Severe combined immunodeficiency


Common variable immunodeficiency
Isolated IgA deficiency
T-cell immunodeficiency
Thymic aplasia (Di George syndrome)

Block in the differentiation of leukemic


cells with prolonged genration time
clonal expansion of the transformed stem
cells + failure of maturation
accumulation of leukemic blast
suppress normal hematopoietic stem cells

Features
Sudden onset (3 months)
Depressed marrow function
Bone pain and tenderness
Generalized lymphadenophaty
Splenomegaly, hepatomegaly
CNS: headache, vomiting

Most common leukemia in children


(80%)
Treatable and potentially curable
Classified according to lymphocytes and
state of maturation
1.
2.
3.
4.
5.

Early B cell
Pre-B cell
Mature B cell
Early T cell
Mature T cell

Acute Non-lymphocytic Leukemia (ANLL)


Most common in adults; >50% 60years old
70% of adults will enter remission with
induction chemo
25-35% of those in remission will have a 5 year

survival rate

BM transplant

Treatment

Selective radiation

Chemotherapy
1.
2.
3.

Induction
Intensification
Maintenance and consolidation

Bone marrow transplant

Insidious onset
Incidental findings during routine exam

Proliferation and accumulation of mature


lymphocytes which are immunologically
incompetent
B cell line (US)
T cell line (Asia)

Hairy cell leukemia

15% of all leukemias


Chromosomal abnormality (Ph1)
Mostly B cell disease
Leukocytosis
Splenomegaly
Hepatomegaly
Lympadenopathy

Bone marrow transplant 5 year survival


for 50-75% of patients

Two distinct phases


Chronic
Last about 3-4 years
Near end accelerated phase: fever, night

sweats, malaise

Acute
2-4 months
Poor prognosis, palliative management

Primary solid tumors of the lymphoid


system
Cancers involving lymphocytes during
maturation or storage in the bone marrow
Third most common malignacy in children

Hodgkins Lymphoma
Disorders primarily involving the lymphoid
tissues
Anatomical spread
Morphological presence of Reed-Sternberg
cells
60-90% cure rate

Manifestations of Hodgkins
A symptoms
Painless progressive enlargement of a single or

group of nodes (neck)


May spread continuously through out the
lymphatic system

B symptoms
Fever, night sweat, weight loss
Fatigue, anemia

Treatment for Hodgkins


Radiation
Chemotherapy

Non-Hodgkins Lymphoma
Involves lymphoid tissue and may spread
to various tissues
Mostly B cell (80%)
Cause may be viral or genetic
EBV
Immunosuppresed patients
AIDS
After organ transplant

Treatment
Early stage radiation
Late stage chemo and radiation
BM transplant

Transfuse for any severe anemia with


physiologic compromise.
Decide early whether transfusion will
be rare or part of therapy.
Avoid long-term complications by
working with your blood bank and
using chelation theraoy.

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