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一名中国男性先天性红细胞生成异常性贫血病例。

A case of congenital dyserythropoietic anemia in a male Chinese.

作者信息

Szeto S C, Ng C S

出版信息

Pathology. 1986 Jan;18(1):165-8. doi: 10.3109/00313028609090849.

Abstract

A 38 yr-old man has chronic non-spherocytic hemolytic anemia which was attributed to glucose-6-phosphate dehydrogenase (G-6PD) deficiency for the past 20 yr. A diagnosis of congenital dyserythropoietic anemia Type II (CDA) was made because of the bone marrow findings and electron microscopic appearance of the peripheral erythrocytes. Congenital dyserythropoietic anemia, though well recognized in Caucasians, has not hitherto been described in Chinese. Erythrocyte G-6PD deficiency has been found to have an incidence of 5.5% in Southern Chinese. The coexistence of CDA Type II and G-6PD deficiency in this patient may be a chance finding or there may be an interaction between the 2 abnormalities in the clinical manifestations of CDA Type II and G-6PD deficiency.

摘要

一名38岁男性患有慢性非球形细胞溶血性贫血,在过去20年中一直归因于葡萄糖-6-磷酸脱氢酶(G-6PD)缺乏。由于骨髓检查结果及外周血红细胞的电子显微镜表现,诊断为Ⅱ型先天性红细胞生成异常性贫血(CDA)。先天性红细胞生成异常性贫血虽在高加索人中已得到充分认识,但迄今尚未见在中国人群中的报道。已发现红细胞G-6PD缺乏在华南地区中国人中的发生率为5.5%。该患者Ⅱ型CDA与G-6PD缺乏并存可能是偶然发现,也可能是这两种异常在Ⅱ型CDA和G-6PD缺乏的临床表现中存在相互作用。

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