Sultan C, Sigaux F, Imbert M, Reyes F
Br J Haematol. 1981 Sep;49(1):11-6. doi: 10.1111/j.1365-2141.1981.tb07191.x.
Eight patients with acute myelodysplasia and myelofibrosis are described. Four cases were secondary to long-term therapy with cytotoxic agents and four 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 four 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 three cases an overt leukaemia developed with marked pleomorphism of blast cells. The nosology of this syndrome is discussed.
本文描述了8例急性骨髓增生异常伴骨髓纤维化患者。其中4例继发于细胞毒性药物的长期治疗，4例为特发性。所有病例均起病急骤，无脏器肿大，伴有严重全血细胞减少。4例骨髓穿刺获取了足够的标本，显示出骨髓增生异常的特征。组织学切片研究表明，每例患者的骨髓均有细胞增多，包括大量营养不良性巨核细胞、成红细胞、粒细胞系未成熟细胞以及难以识别的原始细胞。网状纤维网络总是增加。每例患者病情均迅速恶化死亡。化疗未见改善。3例患者发展为明显的白血病，原始细胞具有明显的多形性。本文对该综合征的疾病分类进行了讨论。
