There is presently no therapeutically or prognostically meaningful staging system for these disorders. Leukemia is considered to be disseminated in the hematopoietic system at diagnosis, even in children with acute myeloid leukemia (AML) who present with isolated chloromas (also called granulocytic sarcomas). If these children do not receive systemic chemotherapy, they invariably develop AML in months or years. AML may invade nonhematopoietic tissue such as meninges, brain parenchyma, testes or ovaries, or skin (leukemia cutis). Extramedullary leukemia is more common in infants than in older children with AML.
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Childhood AML is diagnosed when bone marrow has greater than 20% blasts. The blasts have the morphologic and histochemical characteristics of one of the French-American-British subtypes of AML. It can also be diagnosed by biopsy of a chloroma. For treatment purposes, children with a t(8;21) and less than 20% marrow blasts should be considered to have AML rather than myelodysplastic syndrome.
Remission is defined in the United States as peripheral blood counts (white blood cell count, differential, and platelet count) rising toward normal, a mildly hypocellular to normal cellular marrow with fewer than 5% blasts, and no clinical signs or symptoms of the disease, including in the central nervous system or at other extramedullary sites. Achieving a hypoplastic bone marrow is usually the first step in obtaining remission in AML with the exception of the M3 (acute promyelocytic leukemia [APL]); a hypoplastic marrow phase is often not necessary prior to the achievement of remission in APL. Additionally, early recovery marrows in any of the subtypes of AML may be difficult to distinguish from persistent leukemia; correlation with blood cell counts, clinical status, and cytogenetic/molecular testing is imperative in passing final judgment on the results of early bone marrow findings in AML. If the findings are in doubt, the bone marrow aspirate should be repeated in about 1 week.
Ebb DH, Weinstein HJ: Diagnosis and treatment of childhood acute myelogenous leukemia. Pediatr Clin North Am 44 (4): 847-62, 1997.
Chan GC, Wang WC, Raimondi SC, et al.: Myelodysplastic syndrome in children: differentiation from acute myeloid leukemia with a low blast count. Leukemia 11 (2): 206-11, 1997.
Konopleva M, Cheng SC, Cortes JE, et al.: Independent prognostic significance of day 21 cytogenetic findings in newly-diagnosed acute myeloid leukemia or refractory anemia with excess blasts. Haematologica 88 (7): 733-6, 2003.
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September 04, 2014
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