TY - JOUR
T1 - Acute Megakaryocytic Leukemia (M7) in Children
AU - WINDEBANK, KEVIN P.
AU - TEFFERI, AYALEW
AU - SMITHSON, W. ANTHONY
AU - LI, CHIN YANG
AU - SOLBERG, LAWRENCE A.
AU - PRIEST, JOHN R.
AU - ELLIOTT, STEPHEN C.
AU - de ALARCON, PEDRO A.
AU - WEINBLATT, MARK E.
AU - BURGERT, E. OMER
PY - 1989
Y1 - 1989
N2 - We analyzed the clinical and laboratory features of eight children (median age, 20 months; range, 13 months to 11 years) with acute megakaryocytic leukemia (M7) and compared the findings with those reported in the literature. The diagnosis was supported by ultrastructural examination for platelet peroxidase or immunophenotyping for glycoprotein IIb/IIIa or the von Willebrand factor protein. Two patients had Down's syndrome. Initial findings included anemia (in all patients), thrombocytopenia (in six), myelofibrosis (in three), lytic bone lesions (in two), and pronounced leukocytosis (in one). Stem cell culture studies of peripheral blood specimens revealed an aberrant phenotype of the megakaryocytes in one patient and reversal to a normal pattern after successful therapy. Remission was achieved in seven of the eight patients after aggressive chemotherapy, and four patients remained in remission 27 to 57 months after diagnosis. Three of these four patients underwent allogeneic bone marrow transplantation. M7 leukemia is not infrequent in children younger than 3 years of age, especially in those with Down's syndrome. The availability of monoclonal antibodies specific to restricted antigens of the megakaryocytic lineage has made the diagnosis of M7 leukemia both possible and practical.
AB - We analyzed the clinical and laboratory features of eight children (median age, 20 months; range, 13 months to 11 years) with acute megakaryocytic leukemia (M7) and compared the findings with those reported in the literature. The diagnosis was supported by ultrastructural examination for platelet peroxidase or immunophenotyping for glycoprotein IIb/IIIa or the von Willebrand factor protein. Two patients had Down's syndrome. Initial findings included anemia (in all patients), thrombocytopenia (in six), myelofibrosis (in three), lytic bone lesions (in two), and pronounced leukocytosis (in one). Stem cell culture studies of peripheral blood specimens revealed an aberrant phenotype of the megakaryocytes in one patient and reversal to a normal pattern after successful therapy. Remission was achieved in seven of the eight patients after aggressive chemotherapy, and four patients remained in remission 27 to 57 months after diagnosis. Three of these four patients underwent allogeneic bone marrow transplantation. M7 leukemia is not infrequent in children younger than 3 years of age, especially in those with Down's syndrome. The availability of monoclonal antibodies specific to restricted antigens of the megakaryocytic lineage has made the diagnosis of M7 leukemia both possible and practical.
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U2 - 10.1016/S0025-6196(12)65376-2
DO - 10.1016/S0025-6196(12)65376-2
M3 - Article
C2 - 2531821
AN - SCOPUS:0024803871
SN - 0025-6196
VL - 64
SP - 1339
EP - 1351
JO - Mayo Clinic proceedings
JF - Mayo Clinic proceedings
IS - 11
ER -