23例儿童纯红细胞再生障碍性贫血临床分析

Clinical analysis of 23 patients with pure red cell aplasia

目的探讨儿童单纯红细胞再生障碍性贫血(pureredcellaplasia,PRCA)的发病机制、诊断和治疗。方法对我院1994年至2004年期间收治的23例PRCA患者进行回顾性分析。总结其病因、临床特点及治疗。结果先天性PRCA10例,获得性PRCAl3例,其中5例继发于病毒感染,8例未找到明确病因。实验室检查:血红蛋白及网织红细胞均下降,白细胞、血小板正常。骨髓检查红系增生低下17例,停滞6例。骨髓干细胞培养10例,7例生长不良。染色体检查8例均正常。治疗以单纯激素口服,甲基强的松龙冲击,激素联合丙种球蛋白以及其他免疫抑制剂为主。目前停药9例,小剂量激素维持11例,无效3例。长期应用激素有多毛、骨质疏松、高血压、身材矮小、白内障等副作用。结论儿童PRCA大部分病因不明确,部分病人与病毒感染相关。诊断需依靠血像及骨髓像。肾上腺皮质激素治疗效果良好,联合丙球治疗可获得更好的疗效。病毒感染继发的PRCA是可治愈的,应首先在抗病毒治疗的同时应用免疫抑制剂。长期口服激素需注意其副作用。

Objective We intended to study the pathogenesis, diagnosis and treatment of childhood PRCA. Methods Twenty - three hospitalized patients from 1994 to 2004 were analyzed. We summarized the pathogen, clinical Character and treatment reaction of this disease. Results There were ten Diamond - Blackfan anaemias and thirteen acquired PRCAs（five of them were virus infection associated, the pathogen of others were unknown）. Laboratory examination： CBC showed erythrocytopenia, white cell alnd platelet were normal, The progenitor of erythrocyte was absent in bone marrow. Chromosome test had been done in eight patients. The results were normal. The patients were treated with prednisone, high - dose methylprednisolone or incombination with immunoglobulin and other i mmunosuppressives. Side effects of corticoid （hypertension, osteoporesis, dandiprat, cataract, etc） could be seen in patients who used them for a long time. Conclusions The pathogens of PRCA were not clear. Some of them were induced by virus infection. CBC and bone marrow test were very important for diagnosis of PRCA. The prognosis of most patients was well after treated with corticoid. Combined treatment with corticoid and IVIgG has synergistic effect. Patients infected by virus needed anti - virus treatment and could becured. We should reduce side effects in patients who need oral corticoid for a longtime.