ACUTE MYELODYSPLASIA WITH MYELOFIBROSIS - A REPORT OF 8 CASES
ACUTE MYELODYSPLASIA WITH MYELOFIBROSIS - A REPORT OF 8 CASES
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DOI：
10.1111/j.1365-2141.1981.tb07191.x
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发表时间：
1981-01-01
影响因子：
6.5
通讯作者：
REYES, F
中科院分区：
文献类型：
--
作者：
SULTAN, C;SIGAUX, F;REYES, F
描述了八名患有急性骨髓增生异常和骨髓纤维化的患者。 4 例继发于长期细胞毒药物治疗，4 例为特发性。所有病例均发病突然，无器官肿大和严重全血细胞减少。 4 例患者的骨髓抽吸物获得足够的物质，并显示骨髓增生异常的特征。组织切片研究表明，每例骨髓均呈细胞状，包括大量营养不良的巨核细胞、成红细胞、粒细胞系未成熟细胞和难以识别的母细胞。网状蛋白网络总是增加。在每种情况下，这种疾病都会迅速致命。化疗没有发现任何改善。 3 例患者出现明显的白血病，并伴有明显的母细胞多形性。讨论了该综合征的疾病分类学。
Eight patients with acute myelodysplasia and myelofibrosis are described. Four cases were secondary to long-term therapy with cytotoxic agents and 4 were idiopathic. All cases presented with an abrupt onset of the illness, absence of organomegaly and severe pancytopenia. Bone marrow aspirate yielded adequate material in 4 cases and showed myelodysplasic features. Study of histological sections indicated that the bone marrow was cellular in every case, including numerous dystrophic megakaryocytes, erythroblasts, immature cells of the granulocytic series and blast cells which were difficult to identify. The reticulin network was always increased. In each case the disease was rapidly fatal. No improvement was noted with chemotherapy. In 3 cases an overt leukemia developed with marked pleomorphism of blast cells. The nosology of this syndrome is discussed.