Childhood Leukemias Flashcards by Evelyn Vassiliou-Romano

What is leukemia

Malignant cell - blast

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What blasts do in leukemia

Baby white blood cells that fail to mature

Crowd normal cells

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Classification of leukemia

Acute : sudden onset; death in weeks to months of left untreated

Chronic: insidious (gradual) onset; death in years of left untreated

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Types of childhood leukemia lineage, onset and type

Lymphoid; acute; ALL
Myeloid; acute; AML
Myeloid; chronic; CML

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Children at increased risk for leukemia

Down’s syndrome

Neurofibromatosis type 1

Chromosomal breakage syndromes
Fanconi anemia
Bloom syndrome
Ataxia-telangiectasia

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Incidence of ALL and age

2-5 years
75-80% childhood leukemia
25% of childhood cancer

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Incidence of AML and age

2-5 years
15-20% childhood leukemia
5% all childhood cancers

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Incidence of CML and age

Age none
<5% childhood leukemia
<1% of all childhood cancers

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Survival rate ALL

91.8%

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Survival rate AML

66.4%

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Leukemia pathophysiology

Genetic damage to single bone marrow white blood cell

Uncontrolled proliferation of blasts

Decreased production of normal cells

Accumulation of blasts in body
organs/ tissues ( mostly liver and spleen)

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Acute leukemia (ALL and AML) symptom onset and clinical appearance

1-6 weeks prior to diagnosis (days to several months)

Children with AML often “sicker” than those with ALL at diagnosis

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Acute leukemia clinical presentation symptoms

Pallor
Fatigue
Anorexia
Fever/infection
Bruising/bleeding
Bone/joint abdominal pain
Headache
Vomiting
Visual disturbances

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Acute leukemia clinical presentation: signs

Pallor
Tachycardia
Petechiae/purpura
Evidence of infection

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Acute leukemia clinical presentation: signs- organs affected (blast accumulation)

Lymphadenopathy (swollen lymph nodes)
Hepatosplenomegaly
Cranial nerve palsies (paralysis and involuntary tremors)
Testicular enlargement

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Acute leukemia clinical presentation: signs-

Chloromas
A rare type of cancer that is made up of myeloblasts (a type of immature white blood cell) and forms outside the bone marrow and blood

Leukemia cutis
the infiltration of neoplastic leukocytes or their precursors into the epidermis, the dermis, or the subcutis, resulting in clinically identifiable cutaneous lesions.

Gingival infiltration
represents a 5% frequency as the initial presenting complication of AML

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Diagnostic work up: CBC

WBC: high, low, or normal
Platelets: low
Hgb: low
Differential: neutropenia and blasts
10% of patients have normal CBC

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Can type of leukemia be diagnosed by CBC

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Diagnostic work up: chemistry panel

Potassium, phosphorus and iron acid may be high due to (cell tumor lysis)

Calcium: low (binds with phos)

Creatinine: high due to renal insufficiency due to tumor lysis

Lactate dehydrogenase (LDH)
High. Correlates to tumor burden

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Diagnostic work up: coagulation studies

Presence of coagulopathy
Indicators:
High PTT and PT
Low fibrinogen
High fibrinogen degradation products (FDP)
High d-dimer

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FDP (fibrin degradation products)

Clothing and bleeding at the same time

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Diagnostic work up additional labs

Viral titers:
Varicella
Hepatitis
Cytomegalovirus
Herpes simplex virus
HIV

Quantitative immunoglobulins
used to detect abnormal levels of the three major classes (IgG, IgA and IgM)

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Diagnostic work up : chest X-ray

Related to mediastinal mass
T-cell leukemia subtype — THORAX
present in 5-10% of ALL
Mass may cause respiratory arrest

**always obtain chest X-ray prior to sedating patient for procedure or surgery

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Diagnostic work up: BMA / biopsy

Posterior iliac crest
Marrow
Spongy bone
Cortical bone

Diagnostic work up: bone marrow cells

Morphology Immunophenotyping Cytogenetics Clinical features (antigens)

Bone marrow analysis: morphology

Cellularity Cell lines Blast percentage

Cellularity

Hypocellular Hypercellular

Cell Lines

Erythroid (RBC precursors) Granulocyte (WBC precursors) Megakaryocytic (platelet precursors)

Bone marrow analysis: morphology Cells classified according to

World Health Organization (WHO)

Bone marrow analysis: Immunophenotyping - what does it identify

Markers (antigens) on blast cells

What does Immunophenotyping help to differentiate

ALL vs AML T vs B cell lineage (ALL) Certain subtypes of AML

What is flow cytometry

Using monoclonal antibodies to determine what antigens are on the leukemic cell

Bone marrow analysis: Cytogenetics

How many chromosomes in leukemia cell: hyper and hypoploidy Chromosome structure: deletions and translocations

Diagnostic work up: lumbar puncture (LP)

Standard component of workup for acute leukemia Cell count: WBC and RBC per mL of CSF Cytology: presence or absence of blasts in CSF IT chemo administered during diagnostic LP (usually cytarabine). Prophylactic chemo

Went night a newly diagnosed pediatric leukemia patient have visual disturbances vomiting and a headache

Increased intracranial pressure from blasts

ALL stand for

Acute Lymphoid Leukemia

Lymphoid Cell Lines

1. Pluripotent stem cell 2. Lymphoid progenitor 3. B & T lymphocytes

Types of ALL

Pre-B cell (84%) - precursor B cell T-cell ALL (15%) Mature B cell ALL (1%) - Burkitts ALL

Precursor B Cell ALL

Most common type leukemia Commonly seen in preschoolers

T-cell ALL

High WBC Often seen with mediastinal mass Adolescent males

Mature b-cell ALL (burkitt)

Respond poorly to standard ALL therapy Treatment same as for Burkitt lymphoma

Cytogenetics in ALL

Ploidy: number of chromosomes Diploid Hyperdiploid Hypodiploid

Diploid

Normal 23 pairs of chromosomes for total 46

Hyperdiploid

Extra copies of chromosomes USUALLY FAVORABLE Maybe less chemo More common than hypo

Hyoploidy

Missing copies of chromosomes ALWAYS UNFAVORABLE low percentile with this

Cytogenetics : unfavorable structural change

Ph+ (translocation accelerator )

What types of cancer have possible Ph+ gene

Mostly CML (some ALL)

Childhood ALL: bone marrow rating

M1 M2 M3

M1 bone marrow rating ALL

<5% blasts GOOD!! Means remission

M2 bone marrow rating ALL

5 to <25% blasts in marrow

M3 bone marrow rating ALL

> or equal to 25% blasts BAD

What is the most important prognostic factor in childhood ALL

MRD: minimal residual disease The time it takes to eliminate the bulk of leukemia

MRD can now be tested how instead of a microscope

Blood test. 100x more sensitive than microscope

Measurement of MRD by bone barrow sample is done when

End of induction 3-4 months

At end of induction what is favorable MRD

<.01% or undetectable

At end of induction what is unfavorable MRD

Persistent MRD > or equal to 0.01% for B lineage ALL

3-4 months out what is a favorable MRD

Low MRD <0.01% or undetectable

3-4 months out what is a unfavorable MRD

Persistence of MRD > or equal to 0.01% for both B and T lineage ALL