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骨髓增生异常综合征:来自吉隆坡大学医院的综述

Myelodysplastic syndrome: a review from University Hospital, Kuala Lumpur.

作者信息

Ng S C, Kuperan P, Bosco J, Menaka N

机构信息

Department of Pathology, Faculty of Medicine, University of Malaya, Kuala Lumpur.

出版信息

Singapore Med J. 1990 Apr;31(2):153-8.

PMID:2196685
Abstract

Twenty patients with Myelodysplastic Syndrome (MDS) were diagnosed in University Hospital, Kuala Lumpur over a 5 year period. They were subclassified using the French American British (FAB) criteria. 90% of the patients were above 40 years old and the sex ratio was about equal. The predominant presenting symptom was anaemia and there was paucity of physical signs at presentation. Patients with 'aggressive' subtypes of MDS i.e. refractory anaemia with excess blasts (RAEB), refractory anaemia with excess blasts in transformation (RAEB(-)+) and chronic myelomonocytic leukaemia (CMML) had more frequent thrombocytopenia and neutropenia and their marrow pictures frequently had dysmegakaryopoiesis and dysgranulopoiesis as compared to more the "benign" subtypes i.e. refractory anaemia (RA) and refractory leukaemic anaemia with ringed sideroblasts (RARS). Four patients had leukaemic transformation and all of them came from the 'aggressive' subtypes. The current views on treatment of MDS are discussed.

摘要

在五年期间,吉隆坡大学医院诊断出20例骨髓增生异常综合征(MDS)患者。他们根据法美英(FAB)标准进行了亚分类。90%的患者年龄在40岁以上,性别比例大致相等。主要的首发症状是贫血,就诊时体征较少。与“良性”亚型即难治性贫血(RA)和环状铁粒幼细胞难治性白血病贫血(RARS)相比,MDS“侵袭性”亚型的患者,即伴有过多原始细胞的难治性贫血(RAEB)、转化中的伴有过多原始细胞的难治性贫血(RAEB(-)+)和慢性粒单核细胞白血病(CMML),血小板减少症和中性粒细胞减少症更为常见,其骨髓图像经常出现巨核细胞生成异常和粒细胞生成异常。4例患者发生白血病转化,且均来自“侵袭性”亚型。文中讨论了目前关于MDS治疗的观点。

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