Acute Megakaryocytic Leukemia (M7) in Children

Windebank, Kevin; Tefferi, Ayalew; Smithson, William A.; Li, Chin Yang; Solberg, Lawrence A.; Priest, John R.; Elliott, Stephen C.; Alarcón, Pedro A. de; Weinblatt, Mark E.; Burgert, E. Omer

doi:10.1016/s0025-6196(12)65376-2

Cited by 36 publications

(16 citation statements)

References 52 publications

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“…14 M7 was found to be the major subtype for patients aged 1 to 5 years; half of these cases were associated with Down syndrome, which has been recognized to have a high frequency with M7 AML in patients under age 3 years in Japan 15 and the USA. 16 Another half of M7 was not associated with Down syndrome. M7 AML without Down syndrome has also been recognized to affect young children.…”

Section: Discussionmentioning

confidence: 95%

Clinicopathologic characteristics of leukemia in Japanese children and young adults

2001

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The aim of this study is to clarify the clinicopathologic characteristics of adolescent leukemia in Japan by retrospective analysis. Patients with acute lymphoblastic leukemia (ALL), acute myelogenous leukemia (AML), chronic myelogenous leukemia (CML), and myelodysplastic syndrome (MDS), consecutively diagnosed from 1986 to 1999, were enrolled. A total of 3856 patients from 1 to 15 years of age and 1803 patients from 15 to 29 years of age were eligible for this study. Demographically, the frequency of AML found was almost constant during the teenage years, whereas the frequency of ALL gradually decreased. The relative frequency of CML and MDS apparently started to increase in patients in their late teens. The relative frequency of M3 and t(15;17) gradually increased during adolescence. Among patients aged 1 to 4 years, M7 was the most frequent FAB subtype. Among patients aged 5 to 9 years, M2 and t(8;21) was the most frequent subtype. The percentage of T cell ALL increased in patients 5 to 9 years old, reaching 31.2% in the 20-to 24-year-old age group. The percentage of patients with hyperdiploidy over 50 chromosomes was highest (17.0%) in patients aged 1 to 4 and decreased to 3.9% in the older teens. The percentage of patients with the Ph 1 chromosome increased from 9.9% in teens to 30.0% in patients in their late twenties. When comparing event-free survival (EFS) rates for ALL according to age, the estimated 7-year EFS rate was highest for patients aged 1 to 9 years (65.9%) and intermediate for patients aged 10 to 15 years (48.4%). However, the EFS rate was significantly worse for patients aged 15 to 19 years (19.4%) and 20 to 29 years (17.0%) (P = 0.024). On the other hand, the EFS rate for AML decreased with increasing age, although without statistical significance. The overall survival rates are approximate among all age groups. The results of the study indicate that there are considerable variations in biologic features of leukemia between children and young adults. The prognosis for adolescent leukemia may be improved by introducing pediatric trials, which take into account the prognostic biological features. Leukemia (2001Leukemia ( ) 15, 1256Leukemia ( -1261

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Section: Discussionmentioning

confidence: 95%

Clinicopathologic characteristics of leukemia in Japanese children and young adults

2001

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“…Megakaryoblasts are identified by a positive platelet peroxidase reaction, 21 and by immunophenotyping for glycoprotein IIb/IIIa or the von Willebrand factor protein. 22 These blasts are non-reactive for myeloperoxidase and express stem/progenitor markers CD33, CD34, CD117, erythroid markers CD36 and glycophorin A, the lymphoid antigen CD7 and the megakaryocytic markers CD41 and CD42b. 23–25 Of note, cytogenetic differences between DS and non-DS AMKL include the absence of the translocation t(1;22), and instead, the presence of trisomies involving chromosomes 8 and 1, 7 as well as monosomy 7.…”

Section: Clinical Featuresmentioning

confidence: 99%

Myeloid Leukemia in Down Syndrome

2011

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Although adults with Down syndrome (DS) show a decreased incidence of cancer as compared to individuals without DS, children with DS are at an increased risk of leukemia. Nearly half of these childhood leukemias are classified as acute megakaryoblastic leukemia (AMKL), a relatively rare subtype of acute myeloid leukemia (AML). Here, we summarize the clinical features of myeloid leukemia in DS, review recent research on the mechanisms of leukemogenesis, including the roles of GATA1 mutations and trisomy 21, and discuss treatment strategies. Given that trisomy 21 is a relatively common event in hematologic malignancies, greater knowledge of how the genes on chromosome 21 contribute to DS-AMKL will increase our understanding of a broader class of patients with leukemia.

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“…7 Approximately 1% of AML cases in adults and 5% to 10% in children are M7 AML. 8,9 Its incidence is higher in children with Down syndrome. 10,11 The prognosis for patients with M7 AML is poor.…”

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confidence: 99%

Hematopoietic stem cell transplantation for de novo acute megakaryocytic leukemia in first complete remission: a retrospective study of the European Group for Blood and Marrow Transplantation (EBMT)

Garderet

Labopin

Gorin

et al. 2005

Blood

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Acute megakaryoblastic leukemia (M7 AML) is a highly aggressive disease. We evaluated outcomes in 57 children (11 with Down syndrome) and 69 adults with M7 AML after first complete remission (CR1) following autologous or HLA-identical allogeneic transplantation. Characteristics of the recipients of autologous transplants (38 children, 37 adults) were, respectively: median age, 1.7 and 46 years; non-total body irradiation (non-TBI) conditioning regimen, 97% and 70%; bone marrow as stem cell source, 74% and 43%. Characteristics of the recipients of allogeneic transplants (19 children, 32 adults) were, respectively: median age, 2.8 and 37 years; non-TBI regimen, 63% and 42%; bone marrow as stem cell source, 95% and 69%. Autologous transplantation benefited children more; the relapse rate was high in adults. Results for autologous transplantation were (children and adults, respectively): engraftment, 90% and 100%; 3-year treatmentrelated mortality (TRM) rate, 3% and 8%; relapse rate, 45% and 64%; leukemia-free survival (LFS) rate, 52% and 27%; overall survival (OS) rate, 61% and 30%. After allogeneic transplantation, TRM was fairly low in children and adults, and relapse rates were lower than after autologous transplantation. Results for allogeneic transplantation were, respectively: engraftment, 95% and 90%; TRM, 0% and 26%; relapse rate, 34% and 28%; LFS, 66% and 46%; OS, 82% and 43% IntroductionAcute megakaryoblastic leukemia (M7 AML) is a rare subtype of acute myeloid leukemia (AML) that develops from primitive megakaryoblasts. It was first described by Van Boros and Korenyi in 1931, and it is the seventh AML subtype in the 1985 FrenchAmerican-British (FAB) classification. 1-3 Developments in cytochemistry and immunophenotyping have improved its diagnosis and differentiation from acute myelosclerosis. 4-6 M7 AML has a bimodal age distribution that peaks in early childhood (younger than 3 years) and in adulthood. 7 Approximately 1% of AML cases in adults and 5% to 10% in children are M7 AML. 8,9 Its incidence is higher in children with Down syndrome. 10,11 The prognosis for patients with M7 AML is poor. Although remission rates are not much lower than those for other myeloid leukemia subtypes, the relapse rate is high. Approximately half the patient population achieves complete remission (CR) with conventional chemotherapy, but few patients survive beyond 3 years. [12][13][14][15] Median overall survival (OS) is estimated to be 40 weeks in adults, 15 and the 2-year event-free survival is 14% in children. 16 Outcomes are best in children with Down syndrome. [16][17][18] Postremission therapy must be improved. It has been suggested that autologous and allogeneic bone marrow transplantation (BMT) might offer the best chance of cure for patients in remission, but available clinical data are scant. Published reports are often case reports 19,20 or small clinical series with inconsistent results. 15,16,21,22 The aim of this retrospective study is to evaluate outcomes after hematopoietic stem cell transplantation (HSCT...

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Acute Megakaryocytic Leukemia (M7) in Children

Cited by 36 publications

References 52 publications

Clinicopathologic characteristics of leukemia in Japanese children and young adults

Clinicopathologic characteristics of leukemia in Japanese children and young adults

Myeloid Leukemia in Down Syndrome

Hematopoietic stem cell transplantation for de novo acute megakaryocytic leukemia in first complete remission: a retrospective study of the European Group for Blood and Marrow Transplantation (EBMT)

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