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ACUTE

LEUKEMIA :
What is it?
Leukemia:
Definition

• Leukemias are clonal, neoplastic proliferations


of immature cells of the hematopoietic system,
which are characterized by aberrant or
arrested differentiation.

• Hematopoietic differentiation is controlled by


regulation of genes in the nucleus and when
this regulation apparatus is disrupted, the
consequence can be leukemia's
• Every healthy blood cell starts its life in the
bone marrow as a hematopoietic stem cell.
They are pluripotent meaning they give rise
to both myeloid and lymphoid blood cells
• zz lymphocytes

Granulocytes
Once they mature they leave the bone marrow & travel around the
blood or settle down in tissues and organs like lymph nodes and spleen
All
trans
retinoic
acid
There are
conditions
not
necessarily
AML but
can lead to
AML
Leukemia
• A group of malignant disorders affecting
the blood and blood-forming tissues of
– Bone marrow
– Lymph system
– Spleen
• Occurs in all age groups

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Leukemia
• Results in an accumulation of dysfunctional
cells because of a loss of regulation in cell
division
• Fatal if untreated
– Progressive

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Leukemia
• Often thought of as a childhood disease
• The number of adults affected with
leukemia is 10 times that of children

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Leukemia
Etiology and Pathophysiology
• No single causative agent
• Most from a combination of factors
– Genetic and environmental influences

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Leukemia
Etiology and Pathophysiology
• Associated with the development of
leukemia
– Chemical agents
– Chemotherapeutic agents
– Viruses
– Radiation
– Immunologic deficiencies

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Leukemia
Classification
• Acute versus chronic
– Cell maturity
• Acute: clonal proliferation of immature
hematopoietic cells (the formation of blood or blood
cells )
• Chronic: mature forms of WBC; onset is more
gradual
– Nature of disease onset

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Leukemia
Classification
• Type of white blood cell (WBC)
– Acute lymphocytic leukemia (ALL)
– Acute myelogenous leukemia (AML)
• Also called acute nonlymphoblastic leukemia
(ANLL)
– Chronic myelogenous leukemia (CML)
– Chronic lymphocytic leukemia (CLL)

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Myelogenous Leukemia
• Leukemia characterized by proliferation of
myeloid tissue (as of the bone marrow and
spleen) and an abnormal increase in the
number of granulocytes, myelocytes, and
myeloblasts in the circulating blood

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Leukemia
Presentation: Acute onset

• Short duration of symptoms


• Child Ok until 1 to 2 wks back- suddenly fell ill
• Child symptomatic
• CBC usually abnormal
↑ WBC; ↓Hb; ↓ Plts

• No problem in diagnosis!
These partially developed WBCs interfere with the
development and function of healthy WBC, platelets and RBCs
Chromosomal
changes alter As a
the cells cycle result
Over time Until eventually

Tough to survive
with extra
competition for
nutrients
The most
common cause is
chromosome
Results translocation
in -

BCR-ABL keeps on TK
Premature
When they combine leukocytes
Forms a fusion gene in BM that
spill into
Codes for the protein blood
Sitting next
Activates enzyme

Modified
chromosome PH
Likely
cause is
mutations
These B
lymphocytes
build up in
the blood

The reduced or adverse


functioning of the CLL cells
lack any
identifiable
cytoplasmic
membrane
or nuclear
structure

Philadelphia chromosome in CML and chromosomal defects in CLL


Imatinib mesylate
(Gleevec®)
Dasatinib (Sprycel®)
Nilotinib (Tasigna®).
Leukemia
Presentation: Insidious Onset

• Longer duration of symptoms


• Fever > 2 wks
• Bony pains: as opposed to joint pains
• Loss of wt/appetite
• Child becomes less active

• Problem in diagnosis!
Leukemia
S/S: BM replacement with blasts:

- RBC: Anemia, Pallor, Fatigue

- Platelets: Purpura / Echym./petechiae


Mucosal bleeding

- Leukocyte: Infection, Fever


Leukemia
S/S : Organ infiltration with blasts:

- Hepatomegaly; splenomegaly
- Bony pains
- lung, CNS, testis
Leukemia
When to suspect?
• Unexplained fever > 2 wks
• Recurrent fever with bone pains
• Pallor and fatigue +/- nodes
• Hepatospelnomegaly +/- pethechaie
• Pancytopenia/ Leukocytosis

To do CBC
Leukemia
Why is it important to diagnose Early?
• Delay in diag: Increased mortality/morbidity
– Metabolic problems: Tumor Lysis Syn
– SVC syndrome (partially blocked)
– Increased duration of neutropenia- infection,
pneumonia, sepsis, fungal infection
– Bleeding
• Delay in diag: ↑ tumor load; ↓ prognosis /
Leukemia
Blood Counts

• CBC: Hb, Plt, WBC


MDC
MCV- mean corpuscular volume
RDW- RBC distribution width
• LAP score- leukocyte alkaine phosphatase
• ESR
Leukemia
Blood Tests
• Biochem: BUN / S.Cr
Albumin/Bili/SGOT/SGPT/AlkPo4
Uric A / S. Ca / S.P
S. Electrolytes / LDH
• Blood Group
• HBsAg, HCV, HIV
• Anti HBs Assay
Leukemia
Radiological Investigations

• Xray Chest
• USG Abdomen
• Skeletal Survey – if indicated
• CT Scans – if indicated
Leukemia
CSF Studies

• Cell count
Morphology
Biochemistry
Leukemia
Bone-Marrow Studies
• Morphology
• Cytochemistry
• Surface Markers
• DNA Index
• Cytogenetics: Karyotype, FISH
• DNA+RNA: RT-PCR; RT-PCR multiplex
• DNA Microarray
Leukemias
ALL: T-cell, B-Cell Lineage

AML: M0 Very Undifferentiated


M1 Undifferentiated
M2 Myeloid with differentiation
M3 Acute Promyelocytic
M4 Myelo-Monoblastic
M5 Monoblastic
M6 Erythroblastic
M7 Megakaryoblastic
Leukemia
INITIAL STEPS

FIRST STEP: Early Diagnosis.

SECOND STEP: Proper Diagnosis.

THIRD STEP: Proper Initial Treatment.


Management of Leukemia

(A) Supportive Care


(B) Chemotherapy
(C) Radiotherapy
(D) Biological Therapy
Management of Leukemia
Supportive Care

• Metabolic
• Hyperleukocytosis
• Hematologic
• Surgical
Management of Leukemia
Supportive Care

• Infectious
• Nutritional
• Pain
• Nursing
• Psychological & Emotional
Management of Leukemia
Principles of Chemotherapy

• Treated on Protocol with induction


and consolidation
• Treated with Combination CT
• Dose intensity important
Management of Leukemia
Induction Chemotherapy
• As Many Drugs as possible
• Continuous Chemotherapy

• Aim is to kill all the leukemic cells so that the


Normal Bone-Marrow will recover
• AML: Cytosine arabinaside and daunrobucin
or idarubicin
• ALL: Daunorubicin, Vincristine, Predisone
and Methotrexate intrathecal
Remission Induction Chemotherapy

• Complications: Tumor Lysis Syndrome


Metabolic Problems,
Bleeding
Infection
D.I.C.
Hyperleukocytosis
Thorombosis, Pancreatitis
Hyperglycemia
Management of Leukemia
Consolidation Chemotherapy

• Aim : to consolidate CR
• More intense therapy
• Usually one/two drugs
• HD-MTX; HD-AraC; AraC + VP-16
• MTX: Methotrexate (high dose)
• VP-16 (Etoposide),
• Cytarabine (ara-C)
Management of Leukemia
Maintenance Therapy

• Aim: to take care of Residual Malignant Cells


• Less intense; Oral Therapy
• Usually 6-MP (6-Mercaptopurine)/
Methotrexate.
• For total of 2 to 3 years.
Management of Leukemia
CNS Directed Therapy

• CNS prophylaxis
• Rx of CNS disease

• Intra-thecal: MTX ; TIT (triple intrathecal


therapy - methotrexate, hydrocortisone, and
cytarabine at age-dependent doses.)
• Cranial RT : 2400 / 1800 / 1260 Rads
Management of Leukemia
Transplantation

• Allogenic: related, unrelated


HD CT (High-dose chemotherapy)

• Autologus: HD CT.
Appropriate Rx
SEED
Biology of
Leukemic cells

Rx Pharmacogenetics
Outcome Pharmakokinetics

SOIL
Genotype

Supportive Compliance
Nutrition
care Drug quality
General care
• Pancytopenia treated – PRBC, platelets
• Febrile – antibiotics
• Allopurinol – (decrease acid load) prevent
tumor lysis syndrome
• Neutropenic diet
• Leukostasis (sludging of blood due to
leukaemic white blood cells getting into
blood stream) – exchange transfusion
Leukemias are Curable

PROVIDED THEY ARE….

• diagnosed early
• diagnosed properly
• treated appropriately
Nursing Assessment
• The clinical picture varies with the type of leukemia
as well as the treatment implemented, so the
following must be assessed:
• Health history. The health history may reveal a
range of subtle symptoms reported by the patient
before the problem is detectable on physical
examination.
• Physical examination. A thorough, systematic
assessment incorporating all body systems is
essential.
• Laboratory results. The nurse also must closely
monitor the results of laboratory studies and culture
results need to be reported immediately.
nursing diagnoses for the patient with ALL may include:
• Risk for infection related to overproduction of immature
WBCs.
• Risk for impaired skin integrity related to toxic effects of
chemotherapy, alteration in nutrition, and impaired
immobility.
• Imbalanced nutrition, less than body requirements,
related to hypermetabolic state, anorexia, mucositis, pain,
and nausea.
• Acute pain and discomfort related to mucositis, leukocyte
infiltration of systemic tissues, fever, and infection.
• Hyperthermia related to tumor lysis or infection.
• Fatigue and activity intolerance related to anemia,
infection, and deconditioning.
The major goals for the patient may include:
• Absence of pain.
• Attainment and maintenance of adequate
nutrition.
• Activity tolerance.
• Ability to provide self-care and to cope with
the diagnosis and prognosis.
• Positive body image.
• Education. The nurse should explain the disease course,
treatment, and adverse effects.
• Infection. The nurse should teach the patient and his
family how to recognize symptoms of infection such as
fever, chills, cough, and sore throat.
• Bleeding. The nurse should educate the patient and the
family how to recognize abnormal bleeding through
bruising and petechiae and how to stop it with direct
pressure and ice application.
• Promote good nutrition. The nurse should explain that
chemotherapy causes weight loss and anorexia, so the
patient must be encouraged to eat and drink high-
calorie and high-protein foods and beverages.
• Rehabilitation. The nurse should help establish a
appropriate rehab program for the patient during remission.
• Plan meticulous, supportive care:
• Meningeal leukemia. Watch out for meningeal leukemia (confusion, lethargy,
headache) and know how to manage care after intrathecal chemotherapy.
• Hyperuricemia. Prevent hyperuricemia, a possible result of rapid,
chemotherapy-induced leukemia cell lysis through encouraging fluids to 2000
ml daily, giving acetazolamide and sodium bicarbonate tablets, and allopurinol.
• Infection control. Control infection by placing the patient in a private room and
instituting neutropenic precautions.
• Skincare. Provide thorough skin care by keeping the patient’s skin and perianal
area clean, applying mild lotions and creams to keep skin from cracking and
drying, and thoroughly cleaning skin before all invasive skin procedures.
• Constipation. Prevent constipation by providing adequate hydration, a high-
residue diet, stool softeners, and mild laxatives, and by encouraging walking.
• Mouth ulcers. Control mouth ulceration by checking often for obvious ulcers
and gum swelling, and by providing frequent mouth care and saline rinses.
• Psychological support. Provide psychological support by establishing a
trusting relationship to promote communication.
• Manage stress. Minimize stress by providing a calm, quiet atmosphere that is
conducive to rest and relaxation.
Nursing Interventions and Rationales:
• Initiate bleeding precautions
– Clotting factors are impaired and patients are at a
higher risk of bleeding and bruising
• Assess and manage pain appropriately: Massage,
Positioning, Cool/heat therapy, Aromatherapy, Guided
imagery, Medications as necessary
– Pain can be difficult to control and manage and
medications may be scheduled with PRN measures for
breakthrough pain. Make sure the intervention is
appropriate for the patient and avoid extra stressors
such as movement. Encourage patient to try non-
pharmacological interventions and balance those with
medication for more comprehensive pain control.
• Monitor for signs / symptoms of infection
or sepsis
– Especially during treatment, patients are at
higher risk of developing sepsis. Monitor
closing for signs and symptoms and notify MD
as necessary.
• Promote normothermia
– Progressive hyperthermia may occur as the
body’s response to disease and effects of
treatment. Monitor temperature closely,
especially during chemotherapy.
• Anticipate needs
– Time pain and nausea medications at their peak
according to therapy, chemo and meal times to
increase their effectiveness
• Monitor Intake & Output and
signs/symptoms of dehydration: Skin
turgor Dry mucous membranes,
Capillary refill
– Dehydration and kidney compromise is a
potential complication of disease and
treatment. Encourage hydration and monitor
closely.
• Patient and family education: Symptoms
and disease process, Infection prevention,
Plan of care
– Patients and family members must be
knowledgeable of process and what to expect to
help reduce anxiety and be prepared for
complications as they arise. Educate family
members and caregivers of the importance to
help reduce risk of infection for the patient by
practicing good hand hygiene.
• Avoid risk of infection from procedures:
Foley catheter insertion, Injections, Lines
and tubes
– Lack of sufficient white blood cells damages the
immune system and patients are more prone to
infections. Weight risk versus benefit.
• Promote self care, independence and
ADLs
– Fatigue is a common symptom and can prevent
the patient from participating in self care.
Provide assistance with ADLs as needed and
cluster care to reduce fatigue and promote rest.
Prioritize activities to help conserve energy for
self care.
Evaluation
• Expected patient outcomes may include:
• Shows no evidence of infection.
• Experiences no bleeding.
• Attains optimal level of nutrition.
• Reports satisfaction with pain and comfort
levels.
• Has less fatigue and increased activity.
• Copes with anxiety and grief.
• Absence of complications.
Discharge and Home Care Guidelines
• Most patients cope better when they have an
understanding of what is happening to them.
• Education. Based on the patient’s education, literacy
level, and interest, teaching of the patient and family
should focus on the disease, its treatment, and certainly
the resulting significant risk of infection and bleeding.
• Vascular access device. Management of a vascular
access device can be taught to most patients or family
members, and the nurses may need to provide follow-up
care for the patient.
• Home care services. Coordination of home care services
and instruction can help alleviate anxiety about managing
the patient’s care at home.
• Thank you

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