儿童纯红细胞再生障碍性贫血的临床血液学特征

Clinico-haematological profile of pure red cell aplasia in children.

作者信息

Marwaha R K, Bansal Deepak, Trehan Amita, Marwaha Neelam, Varma Neelam

机构信息

Division of Pediatric Hematology-Oncology, Advanced Pediatric Center, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

出版信息

J Trop Pediatr. 2002 Apr;48(2):113-6. doi: 10.1093/tropej/48.2.113.

DOI:10.1093/tropej/48.2.113

PMID:12022425

Abstract

Pure red cell aplasia (PRCA) is a rare disorder, characterized by isolated failure of erythropoiesis. The clinico-haematological profile of 16 patients with PRCA is presented in this communication. Fourteen patients had Diamond-Blackfan anaemia (DBA), one had transient erythroblastopenia of childhood, and one patient had PRCA secondary to carbamazepine. Physical abnormalities were observed in 50 per cent of patients with DBA. Of the nine patients with DBA who were administered prednisolone and had a regular follow-up, four (44.4 per cent) had no response, three (33.3 per cent) responded fully, and two (22.2 per cent) were steroid dependent.

摘要

纯红细胞再生障碍性贫血（PRCA）是一种罕见的疾病，其特征为单纯的红细胞生成障碍。本文介绍了16例PRCA患者的临床血液学特征。14例患者患有先天性纯红细胞再生障碍性贫血（DBA），1例患有儿童期短暂性红细胞生成减少症，1例患者的PRCA继发于卡马西平。50%的DBA患者存在身体异常。在接受泼尼松龙治疗并定期随访的9例DBA患者中，4例（44.4%）无反应，3例（33.3%）完全缓解，2例（22.2%）对类固醇依赖。