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[长期骨髓增生异常综合征转化为急性白血病]

[Long-term myelodysplastic syndrome transformed into blastic leukemia].

作者信息

Dimova R, Apostolov P, Popova L

出版信息

Vutr Boles. 1988;27(4):99-103.

PMID:3213032
Abstract

A very rare case of myelodysplastic syndrome of long duration transformed into acute leukemia is reported. The syndrome presented with refractory anemia with ring sideroblasts and after eight years of favourable course abruptly turned into acute myelomonoblastic leukemia with a rapid fatal outcome caused by gram-negative sepsis. The disappearance of the ring-sideroblasts from the bone marrow is pointed out as a bad prognostic sign. Treatment of the myelodysplastic syndrome with small doses of cytosine arabinoside is discussed as well as the nonobligatory passing through the stage of a gradual increase of the blast cells before the transformation into acute leukemia.

摘要

报告了一例病程漫长的骨髓增生异常综合征转化为急性白血病的罕见病例。该综合征表现为伴有环形铁粒幼细胞的难治性贫血,在八年的良好病程后突然转变为急性粒单核细胞白血病,最终因革兰氏阴性菌败血症迅速死亡。骨髓中环形铁粒幼细胞的消失被指出是不良预后的标志。文中还讨论了用小剂量阿糖胞苷治疗骨髓增生异常综合征,以及在转化为急性白血病之前并非必须经过原始细胞逐渐增多的阶段。

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