AML—Nejm 1999;341:1051

Pathophys:Perhaps a defect in a maturation stimulator so cells don't die as normally would

Increased incidence in patients treated for Hodgkin's, multiple myeloma, Waldenström's, and ovarian cancers treated with radiation, MOPP (4% at 10 yr get it—Ann IM 1970;72:693), and alkylating agents like melphalan, chlorambucil, thiotepa, and cyclophosphamide (Nejm 1990;322:1, 7); may be preceded by a myelodysplastic syndrome assoc w somatic chromosomal deletions. 2.4 cases/100 000/yr in US; 12.6/100 000/yr over age 65 yr.

Sx:Weakness, bleeding, fever, infections

Si:Anemia; petechiae and ecchymoses (83%); sternal tenderness, lymphadenopathy, hepatosplenomegaly; testicular, skin, meningeal, gum and perianal infiltration, esp with monocytic types

Grim, though still 10% 5-yr survivals; grimmer if alkylating agent-induced (Ann IM 1980;93:133), preceded by myelodysplasia, or if monomyelocytic

CNS involved (7%); sepsis always; significant bleeding

Lab:

Chem: Elevated B₁₂, uric acid, phosphate; low calcium with rx due to PO₄ released by dead cells

Hem:Anemia; decreased platelets; smear shows blasts, if >200 000 needs leukophoresis. Marrow shows blasts >30%; Auer rods sometimes and nucleoli in myeloblasts

Immunol:Circulating immune complexes correlate with worse prognosis (Nejm 1982;307:1174)

Rx:(www.nccn.org)

Induction w cytarabine + daunorubicin: if decide to do, takes 1 mo in hospital, make aplastic and septic usually, 65% chance of inducing, 6-14 mo remission. In a small subset (10%) (promyelocytic), tretinoin (all-trans retinoic acid) matures the leukemic clone (Nejm 1997;337:1021, 1993;329:177)

Maintenance: marrow transplant with autologous marrow, or HLA-matched sibling, or unrelated donor, as good as (44% >4-yr survival) continued intensive chemotherapy?