Pure red cell aplasia: Clinical features and treatment results in 16 cases |
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Authors: | Y L Kwong K F Wong R H S Liang Y C Chu L C Chan T K Chan |
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Institution: | (1) Division of Hematology and Oncology, University Department of Medicine, Professorial Block, Queen Mary Hospital, Pokfulam Road, Hong Kong, HK;(2) Department of Pathology, Queen Elizabeth Hospital, Hong Kong, HK;(3) Department of Medicine, Queen Elizabeth Hospital, Hong Kong, HK;(4) Department of Pathology, Queen Mary Hospital, Hong Kong, HK |
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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.
Received: 11 September 1995 / Accepted: 1 January 1996 |
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Keywords: | Pure red cell aplasia Treatment Steroids Cyclosporine |
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