Acute megakaryocytic leukemia: the Eastern Cooperative Oncology Group experience.
Acute megakaryocytic leukemia: the Eastern Cooperative Oncology Group experience.
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DOI：
10.1182/blood.v96.7.2405.h8002405_2405_2411
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发表时间：
2000-10
期刊：
影响因子：
20.3
通讯作者：
Martin S. Tallman;D. Neuberg;John M. Bennett;Christopher J. François;E. Paietta;P. H. Wiernik;Gordon W. Dewald;P. Cassileth;Martin M. Oken;Jacob M. Rowe
中科院分区：
文献类型：
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作者：
Martin S. Tallman;D. Neuberg;John M. Bennett;Christopher J. François;E. Paietta;P. H. Wiernik;Gordon W. Dewald;P. Cassileth;Martin M. Oken;Jacob M. Rowe
急性巨核细胞白血病（AMegL）是一种罕见的急性髓系白血病（AML）亚型，由原始巨核母细胞进化而来。由于其罕见性和过去缺乏精确的诊断标准，很少有用现代疗法治疗的成人系列报道。1984年至1997年间，1649例新诊断的AML患者中有20例（1.2%）被发现患有AMegL。中位年龄为42.5岁（18-70岁）。骨髓纤维化，通常广泛存在于骨髓。在进行细胞遗传学研究的8例患者中，3号染色体异常最为常见。最一致的免疫表型发现是在5例患者的母细胞中缺乏髓过氧化物酶。在最典型的3例中，白血病细胞除缺乏髓过氧化物酶或与淋巴性白血病一致的抗原外，还具有1至2种血小板特异性抗原阳性。髓过氧化物酶和选择性t细胞抗原（CD7和/或CD2）以外的髓系抗原经常表达。所有病例的诱导治疗包括蒽环类药物和阿糖胞苷。20例患者中有10例（50%）达到完全缓解（CR）。两名患者仍然存活，其中一名患者在160多个月的CR中存活。耐药性疾病是诱导失败的原因，但3例除外。中位CR持续时间为10.6个月（1-160+个月）。所有患者的中位生存期为10.4个月（范围1-160多个月）。虽然有一半的患者达到了CR，但长期预后极差，主要是由于耐药疾病。需要新的治疗策略。
Acute megakaryocytic leukemia (AMegL) is a rare subtype of acute myeloid leukemia (AML) evolving from primitive megakaryoblasts. Because of its rarity and the lack of precise diagnostic criteria in the past, few series of adults treated with contemporary therapy have been reported. Twenty among 1649 (1.2%) patients with newly diagnosed AML entered on Eastern Cooperative Oncology Group (ECOG) trials between 1984 and 1997 were found to have AMegL. The median age was 42.5 years (range 18-70). Marrow fibrosis, usually extensive, was present in the bone marrow. Of the 8 patients who had cytogenetic studies performed, abnormalities of chromosome 3 were the most frequent. The most consistent immunophenotypic finding was absence of myeloperoxidase in blast cells from 5 patients. In the most typical 3 cases, the leukemic cells were positive for one to 2 platelet-specific antigens in addition to lacking myeloperoxidase or an antigen consistent with a lymphoid leukemia. Myeloid antigens other than myeloperoxidase and selected T-cell antigens (CD7 and/or CD2) were frequently expressed. Induction therapy included an anthracycline and cytarabine in all cases. Complete remission (CR) was achieved in 10 of 20 patients (50%). Two patients remain alive, one in CR at 160+ months. Resistant disease was the cause of induction failure in all but 3 patients. The median CR duration was 10.6 months (range 1-160+ months). The median survival for all patients was 10.4 months (range 1-160+ months). Although half of the patients achieved CR, the long-term outcome is extremely poor, primarily attributable to resistant disease. New therapeutic strategies are needed.