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先天性纯红细胞再生障碍性贫血:6例报告。

Diamond-Blackfan anemia: report of 6 cases.

作者信息

Manglani Mamta, Lokeshwar M R, Sharma Ratna

机构信息

Division of Pediatric Hematology-Oncology, Department of Pediatrics, L.T.M.M. College and General Hospital, Sion, Mumbai 400 050, India.

出版信息

Indian Pediatr. 2003 Apr;40(4):355-8.

Abstract

Diamond-Blackfan anemia is a rare congenital hypoplastic anemia. We report 6 children diagnosed as Diamond-Blackfan anemia at our clinic. All had severe pallor at presentation, with mild hepatomegaly and just palpable spleen in one child. Thumb anomaly was present in one of them. All of them had macrocytic or normocytic anemia with reticulocytopenia, and bone marrow examination revealed marked erythroid hypoplasia. All of them were treated with oral steroids with a good response.

摘要

先天性纯红细胞再生障碍性贫血是一种罕见的先天性再生障碍性贫血。我们报告了在我们诊所诊断为先天性纯红细胞再生障碍性贫血的6名儿童。所有患儿就诊时均有严重苍白,1名儿童有轻度肝肿大,脾脏仅可触及。其中1名患儿有拇指异常。所有患儿均有大细胞性或正细胞性贫血伴网织红细胞减少,骨髓检查显示明显的红系造血减低。所有患儿均接受口服类固醇治疗,反应良好。

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