Acute megakaryoblastic leukemia in children: Diagnosis and management challenges in resource-poor countries

Abstract

Acute Megakaryoblastic Leukemia (AMKL) is a rare subtype of acute myeloid leukemia classified as AML M7 by the French–American–British (FAB) Leukemia Cooperative Study Group. We reviewed the records of children aged less than 18 years with AMKL during the period from January 2003 to December 2010. The diagnosis of AMKL was established on the basis of the FAB criteria and confirmed by immunophenotyping. Eight cases were assigned for this period. The incidence rate of AMKL was 5.5% of AML. The median age at the time of diagnosis was 1 year. The karyotype presented translocation (1, 22) in two cases. One girl had Down syndrome (DS). Treatment included two inductions based on daunorubicin and cytosine arabinoside (7+3) followed by two consolidations with high dose of cytosine arabinoside (16 g/m2). Complete remission (CR) was achieved in 3 patients (43%). One patient underwent allogeneic stem cell transplant (SCT) and is still enjoying continuous CR 5 years after treatment. Other patients died from failure or relapse. In this small series from a single institution, reported incidence of AMKL is underestimated probably due to the difficulties of diagnosis. The prognosis was poor, particularly because of unavailability of intensified chemotherapy treatment and allogeneic SCT.