Etiology

The etiology of acute leukemia is unknown. The following factors are important in the pathogenesis of leukemia:

1. Ionizing radiation.

3. Drugs (e.g., use of alkylating agents either alone or in combination with radiation therapy increases the risk of AML).

4. Genetic considerations:

a. Identical twins—If one twin develops leukemia during the first 5 years of life, the risk of the second twin developing leukemia is 20%.

*AML and ANLL are used interchangeably in this chapter and refer to the same disorder.

b. Incidence of leukemia in siblings of a leukemia patient is four times greater than that of the general population.

c. Chromosomal abnormalities:

Group

Risk

Time interval

Trisomy 21 (Down syndrome)

1 in 95

<10 years of age

Bloom syndrome

1 in B

<30 years of age

Fanconi anemia

1 in 12

<16 years of age

d. Increased incidence with the following genetically determined conditions:

(1) Congenital agammaglobulinemia

(2) Poland syndrome

(3) Shwachman-Diamond syndrome

(4) Ataxia telangiectasia

(5) Li-Fraumeni syndrome (germline p53 mutation)—the familial syndrome of multiple cancers in which acute leukemia is a component malignancy

(6) Neurofibromatosis

(7) Diamond-Blackfan anemia

(8) Kostmann disease.

Most cases of leukemia do not stem from an inherited genetic predisposition but from somatic genetic alterations.

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