Pure red cell aplasia: clinical features and treatment results in 16 cases

Y L Kwong, Yok‐Lam Kwong, K. F. Wong, R H Liang, Raymond Liang, Y. C. Chu, Sy Ha, L C Chan, T. K. Chan
Annals of Hematology · 1996 · vol. 72(3) , pp. 137–140 · doi:10.1007/s002770050151 · PMID:8766255 · W2019430928
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This study analyzed clinical features and treatment outcomes in 16 Chinese patients with pure red cell aplasia, finding that conventional corticosteroids, cyclosporin A, and thymectomy yielded variable remission rates while other therapies proved ineffective.

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Abstract

Pure red cell aplasia (PRCA) is a rare hematological disease characterized by selective marrow erythroid aplasia. We report the clinical features and treatment results of 16 Chinese patients with PRCA. Nine (56%) cases were not associated with any underlying disorders and were considered idiopathic, while seven patients (44%) had associated diseases, three involving the thymus, two with T large granular lymphocyte leukemia (T-LGLL), and one each with Stevens-Johnson syndrome and acute hepatitis A. Conventional-dose corticosteroid therapy resulted in complete remission in three of 13 patients. Cyclosporin A was used in six patients. There were three complete and one partial remissions. High-dose methylprednisolone was ineffective in four patients who failed conventional-dose corticosteroids but achieved complete remission in one patient with thymoma who did not respond to thymectomy. Antithymocyte globulin was used in four patients, resulting in partial remission in only one patient with concomitant T-LGLL. Intravenous gamma globulin and danazol were ineffective in three patients. Thymectomy was performed in two patients, with one patient remitting. This is the largest series of PRCA reported in an oriental population. Our results indicate that treatment of PRCA may still be problematic and better therapeutic strategy will have to be defined.

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MeSH descriptors

Red-Cell Aplasia, Pure Red-Cell Aplasia, Pure Adrenal Cortex Hormones Adrenal Cortex Hormones Adult Aged Antilymphocyte Serum Antilymphocyte Serum Blood Transfusion Cyclosporine Cyclosporine Female Humans Male Methylprednisolone Methylprednisolone Middle Aged Red-Cell Aplasia, Pure Remission Induction Thymectomy

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