CHILDHOOD LEUKEMIAS

• The most common childhood cancers (33%).

• It represents a clonal expansion & arrest at a specific stage of normal myeloid & lymphoid
hematopoiesis.
• Types:

o Acute (97%):
1. Lymphoblastic (ALL) 75%.
2. Myeloid (AML) 20%.
3. Undifferentiated (AUL) <0.5%.
4. Mixed lineage (AMLL).

o Chronic (3%):
1. Myeloid.
2. Lymphoblastic (very rare in pediatrics).

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 ACUTE LYMPHOBLASTIC LEUKEMIA
DEFINITION • Abnormal growth & proliferation of
immature lymphocytes
(lymphoblasts) within the BM.
LYMPHOMA
• Abnormal growth & proliferation of
mature lymphocytes within the
lymphatic system which includes LNs,
spleen, thymus, tonsils & BM.

EPIDEMIOLOGY • The most common childhood cancer • The third most common childhood
(25%). cancer (20%) after leukemias (33%) &
• 75% of childhood leukemias. brain tumors (25%).
AGE “Peak inc.” • 2-10 years (10% in infants < 1 year). • 5-15 years (for NHL).
SEX • M:F ratio is 2:1 (with exception of • Males are more affected than
infant leukemia where there is a females.
female predominance.
SOCIAL DIST. • Higher incidence among middle & high socio-economic classes.
GEOGRAPHIC • Highest incidence in Egypt & USA • Higher incidence in blacks than
DIST. (whites). whites (difference in susceptibility or
• Intermediate in most European environmental exposure).
countries.
• Lowest in UAS (blacks), India &
Kuwait.

ETIOLOGY
Unknown but may be due to many factors:
Genetic factors Evidences are: Evidences are:
• Higher incidence among: • Higher incidence among:
1. Siblings of affected children. 1. …
2. Children with down syndrome.
3. Monozygotic twins.
• Occurrence of familial leukemia. • Occurrence of familial lymphoma.
Viral infection As: As:
1. EBV in ALL (L3). 1. EBV.
2. Endemic burkitt’s lymphoma, Human 2. Human T lymphocytic viruses.
T lymphocytic viruses I & II & 3. HIV.
retroviruses in T-cell leukemia.
Environmental • Exposure to ionizing radiation induces chromosomal aberrations, interferes
factors with immunologic defenses & predisposes to malignancy (may cause acute
leukemia).
Chemical • Chronic exposure to: • Chronic exposure to:
carcinogens 1. Benzene, herbicides, pesticides. 1. …
2. Drugs as chemotherapy & 2. …
cholarmphenicol. • Hydantion may be associated with
• Maternal use of contraceptives, pseudo-lymphomas which resolve
cigarettes & alcohol. when the drug is discontinued.
Immune • Children with immune deficiency diseases. have an ↑ risk of …
deficiency • Patients receiving immunosuppressive drugs.
• Patients with autoimmune diseases & organ transplants. (ALL or lymphoma)

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 D.D.

1. AML:
o Morphology, immuno-pheno-typing & cytogenetic study of BM aspirate can differentiate.

2. Leukemic phase of lymphoma:

o …

3. Aplastic anemia: (due to inhibition of normal hematopoietic progenitors by leukemic cells)

o BM biopsy can differentiate.

4. Small round cell tumors: (as neuro-blastoma & retino-blastoma)

o May mimic ALL by clinical & laboratory features when infiltrate BM.
o Demonstration of the 1ry tumor can differentiate.

5. Some infectious diseases: (as IMN)

o May mimic ALL due to fever, lymphadenopathy, HSM, blood cytopenia & atypical lymphocytes.
o Morphology of atypical lymphocytes & cytogenetic study of BM aspirate can differentiate.

6. Osteomyelitis & rheumatologic diseases: (due to bone pain & arthralgia)

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 PROGNOSTIC FACTORS OF ALL
1. AGE:
• Prognosis is poor when age is < 2 years & > 10 years at diagnosis and worst in infant < 1 year.

2. RACE: Higher relapse rate in boys than girls.

3. RACE: Higher relapse rate in black children due to:

1. Higher incidence of high initial WBCs count, mediastinal mass & L2 morphology.
2. Lower socio-economic status.

4. ONSET OF LEUKEMIA:
• The slower the onset, the more durable the remission that follows institution of therapy.

5. LEUKEMIC CELL BURDEN:

• Assessed by evaluation of extra-medullary disease as the degree of HSM & lymph-adenopathy.

6. NUTRITIONAL STATUS:
• Malnourished children have less tolerance & receive sub-optimal doses of chemotherapy.

7. CNS INVOLVEMENT:
• CNS infiltration is associated with lower remission induction, higher relapse rate & shorter survival.

8. Hb CONCENTRATION:
• Hb < 10 gm/dL is associated with higher remission induction, lower relapse rate & longer survival.
• Normal Hb level is associated with bulky extra-medullary disease & high percentage of blasts.

9. WBCs COUNT: High initial WBCs count is associated with bulky extra-medullary disease & high risk
of CNS or testicular relapses.

10. PLATELETS COUNT:

• Platelets count < 20.000/mm3 is associated with shorter remission.
• Leukemia associated with petechial hemorrhages may predispose to CNS or testicular relapses.

11. LDH: High LDH level is associate with shorter remission.

12. BLAST MOTPHOLOGY: L1 have more favorable prognosis than L2 - L3 have the worst prognosis.

13. IMMUNOPHENOTYPING:
• B cell have the worst prognosis.
o Early pre B have more favorable prognosis than pre B - Mature B have extremely poor prognosis.
• T cell have poor prognosis - Early T have more favorable prognosis than mature T.

14. RESPONSE TO TREATMENT:

• Failure to reduce leukemic mass rapidly permits the emergence of clones of lymphoblasts resistant
to drugs.
• So early responders with < 5% blasts in BM on day 7 have the best DFS (Disease Free Survival).

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