UNLABELLED:Pure red cell aplasia (PRCA) is an uncommon disorder, characterized by transfusion dependent anemia, reticulocytopenia with selective aplasia or paucity of erythroid cells in bone marrow. There are only a few large series of PRCA reported in literature. This is the largest single center series of PRCA from India. OBJECTIVE:To evaluate the utility of Immunohistochemistry with Glycophorin A on bone marrow biopsies in quantitating the cut-off percentage of erythroid blasts required for diagnosis, as the upper cut-off percentage of erythroblasts for establishing a diagnosis of PRCA is still not clear. METHODS:The clinical data were obtained from patients' case files. Immunohistochemistry with Glycophorin A was performed using an immunoperoxidase technique and percentage of Glycophorin A positive cells of all nucleated cells was calculated by two independent observers. RESULTS:In our study, bone marrow aspirates showed a variable percentage of erythroblasts ranging from 2 to 12% (mean 6.3%) in children and from 1 to 8% (mean 4.6%) in adults on Giemsa smears. Immunohistochemistry (IHC) with Glycophorin A showed a mean positive cell % of 8.2 (range 2-16%) and 6.8 (1-9%) in pediatric and adult respectively against a mean of 28% (range 21-39%) in idiopathic thrombocytopenia (ITP) cases. Treatment with prednisone showed good response in a majority of both adults and childhood PRCA. Cyclosporine was found to be a good alternative in prednisone non-responders. Thymectomy was beneficial in patients with thymoma. CONCLUSION:A higher percentage of erythroid cells (>5%) does not exclude a diagnosis of PRCA in an appropriate clinical setting and therefore can be managed as PRCA.

译文

纯净红细胞发育不全(PRCA)是一种罕见疾病,其特征为输血依赖性贫血,网状细胞减少伴选择性增生或骨髓中的红系细胞稀少。文献中仅报道了一些大型的PRCA系列。这是印度最大的PRCA单中心系列。
目的:评估糖蛋白A的免疫组织化学在骨髓活检中定量诊断所需要的红系胚细胞的截止百分比的实用性,因为尚无法确定诊断PRCA的红细胞的最高截止百分比。
方法:临床资料来自患者的病例档案。使用免疫过氧化物酶技术用糖蛋白A进行免疫组织化学,并由两名独立的观察员计算所有有核细胞中糖蛋白A阳性细胞的百分比。
结果:在我们的研究中,在Giemsa涂片上,骨髓抽吸物显示儿童中成纤维细胞的百分比可变,成人为2%至12%(平均6.3%),成人为1%至8%(平均4.6%)。糖皮质激素A的免疫组织化学(IHC)显示,儿科和成人的平均阳性细胞百分比分别为8.2(范围为2-16%)和6.8(1-9%),而特发性疾病的平均阳性细胞率为28%(范围为21-39%)血小板减少症(ITP)病例。泼尼松治疗在大多数成人和儿童PRCA中均显示出良好的反应。在强的松无反应者中发现环孢菌素是一个很好的选择。胸腺切除术对胸腺瘤患者有益。
结论:较高比例的红系细胞(> 5%)不能排除在适当的临床环境中诊断出PRCA,因此可以作为PRCA进行管理。

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