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骨髓增生异常综合征:临床与血液学特征分析

Myelodysplastic syndromes: an analysis of clinical and haematological features.

作者信息

Tan Y O, Kueh Y K

出版信息

Ann Acad Med Singap. 1984 Jul;13(3):463-7.

PMID:6517513
Abstract

A retrospective analysis of 24 cases of myelodysplastic syndromes was performed between 1979-1984. There were 16 men and 8 women with a mean age of 69 years and 65 years respectively. The most common haematological features at initial presentation were refractory macrocytic anaemia, reticulocytopenia and associated with either bicytopenia or pancytopenia. The bone marrow was often cellular with megaloblastoid erythropoiesis and in 7 patients (29%), there were ring sideroblasts. Often there was associated dysgranulopoiesis and dysthrombopoiesis Myelodysplastic syndromes appear morphologically to be a heterogenous entity. An attempt to classify them according to the recent proposal of the French-American-British (FAB) Co-operative Group was made. Transformation into acute non-lymphocytic leukaemia occurred in 6 patients (25%). The mean interval between diagnosis and transformation to acute leukaemia was 13 months (range 3-30 months). Acute leukaemia in these patients were refractory to conventional chemotherapy. There were 14 deaths (58%) observed during the period and the common causes of deaths were acute leukaemia, infections and haemorrhage. At present there is no specific or generally effective therapy for this haematologic entity.

摘要

1979年至1984年间对24例骨髓增生异常综合征患者进行了回顾性分析。其中男性16例,女性8例,平均年龄分别为69岁和65岁。初诊时最常见的血液学特征为难治性大细胞贫血、网织红细胞减少,并伴有双系血细胞减少或全血细胞减少。骨髓通常细胞增多,伴有巨幼样红细胞生成,7例患者(29%)出现环形铁粒幼细胞。常伴有粒细胞生成异常和血小板生成异常。骨髓增生异常综合征在形态学上似乎是一种异质性疾病。已尝试根据法美英(FAB)协作组最近的提议对其进行分类。6例患者(25%)转化为急性非淋巴细胞白血病。诊断至转化为急性白血病的平均间隔时间为13个月(范围3至30个月)。这些患者的急性白血病对传统化疗无效。在此期间观察到14例死亡(58%),常见死亡原因是急性白血病、感染和出血。目前对于这种血液学疾病尚无特效或普遍有效的治疗方法。

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