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奥厄小体阳性的骨髓生成异常综合征

Auer rod positive dysmyelopoietic syndrome.

作者信息

Weisdorf D J, Oken M M, Johnson G J, Rydell R E

出版信息

Am J Hematol. 1981 Dec;11(4):397-402. doi: 10.1002/ajh.2830110408.

DOI:10.1002/ajh.2830110408
PMID:6949477
Abstract

Twenty-five patients with refractory anemia with excess blasts (RAEB) were studied. Five of these patients showed Auer rods in their myeloblasts, but met other criteria for RAEB. Median survival of the Auer rod-positive group was 14 months (range 2-27) from diagnosis with survival of 7 months after Auer rods were first observed. Median survival for the Auer rod-negative group was 12 months. Two patients developed overt acute leukemia, both from the Auer rod-negative group. The clinical course of Auer rod-positive RAEB, like that of Auer rod-negative RAEB, was one of progressive bone marrow failure complicated by infection, serious bleeding and the development of absolute transfusion requirement. These findings suggest that Auer rod-positive RAEB is a morphologic variant dysmyelopoietic syndrome that may pursue a similar clinical course to Auer rod-negative disease. Formulation of a separate treatment approach for those RAEB patients who possess Auer rods would appear ill advised.

摘要

对25例伴有过多原始细胞的难治性贫血(RAEB)患者进行了研究。其中5例患者的原始粒细胞中可见奥氏小体,但符合RAEB的其他标准。奥氏小体阳性组从诊断起的中位生存期为14个月(范围2 - 27个月),从首次观察到奥氏小体起生存期为7个月。奥氏小体阴性组的中位生存期为12个月。2例患者发展为明显的急性白血病,均来自奥氏小体阴性组。奥氏小体阳性的RAEB的临床病程,与奥氏小体阴性的RAEB一样,是进行性骨髓衰竭并伴有感染、严重出血以及出现绝对输血需求。这些发现提示,奥氏小体阳性的RAEB是一种形态学变异的骨髓造血异常综合征,其临床病程可能与奥氏小体阴性疾病相似。为那些有奥氏小体的RAEB患者制定单独的治疗方法似乎是不明智的。

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