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Acute megakaryoblastic leukemia in children identified by immunological marker studies
A Hirt1, A R Luethy, B Mueller
1Department of Pediatrics (Inselspital), University of Bern, Switzerland.
Abstract:
Acute megakaryoblastic leukemia (AMkL), defined by the presence of the platelet-associated glycoprotein IIb/IIIa complex on malignant cells, was diagnosed in 4 (4%) of 103 consecutive children with untreated acute leukemia or 4 (21%) of 19 children with acute nonlymphoblastic leukemia (ANLL). Particular features in the four children with AMkL were an age below 12 months at diagnosis (two patients), the absence of a significant hepatosplenomegaly (three patients), a leukocyte count below 20 x 10(9)/L with only a few blast cells in the peripheral blood (four patients), a technically difficult bone marrow aspiration (three patients), the presence of many megakaryocytes in marrow particles (two patients), and an inconclusive cytochemistry (four patients). The four children with AMkL were treated according to protocols for ANLL and a complete remission was obtained in all patients. One patient died from relapse after 3 months, one patient is a long-term survivor (38+ months), and two patients still on chemotherapy are disease-free for 11+ and 13+ months.
Insights
Acute megakaryoblastic leukemia (AMkL) in children is rare but presents unique diagnostic features. Early treatment according to acute non-lymphoblastic leukemia (ANLL) protocols shows promising remission rates.
Area of Science:
- Pediatric Oncology
- Hematology
- Cell Biology
Background:
- Acute megakaryoblastic leukemia (AMkL) is a subtype of acute leukemia characterized by the presence of platelet-associated glycoprotein IIb/IIIa complex on malignant cells.
- AMkL is uncommon in children, making its diagnostic features and treatment outcomes less understood compared to other acute leukemias.
Purpose of the Study:
- To identify the diagnostic characteristics of AMkL in children.
- To evaluate the treatment response and outcomes for pediatric AMkL patients.
Main Methods:
- Retrospective analysis of 103 children with untreated acute leukemia.
- Detailed examination of clinical, laboratory, and bone marrow findings in four diagnosed AMkL cases.
- Treatment of AMkL patients using protocols for acute non-lymphoblastic leukemia (ANLL).
Main Results:
- AMkL was diagnosed in 4% of all childhood acute leukemia cases and 21% of acute non-lymphoblastic leukemia (ANLL) cases.
- Key features in pediatric AMkL included young age at diagnosis (<12 months), absence of significant hepatosplenomegaly, low leukocyte count, difficult bone marrow aspiration, and inconclusive cytochemistry.
- All four AMkL patients achieved complete remission with ANLL-directed therapy, with one long-term survivor.
Conclusions:
- Pediatric AMkL exhibits distinct clinical and laboratory features that can aid in its diagnosis.
- Standard ANLL treatment protocols appear effective in achieving remission for childhood AMkL, warranting further investigation into optimal therapeutic strategies.