[Good's syndrome complicated with pure white cell aplasia].

Le résumé fourni par la source

A 58-year-old man was admitted to our department with a fever that started 1 week prior. His white blood cell count was 600/µl, with 0% neutrophils, and erythrocyte and platelet counts were in the normal range. The bone marrow was hypoplastic with 5.8% myeloblasts, but the number of granulocytes differentiated from promyelocytes was markedly decreased, with an M/E ratio of 0.18. The erythroblasts and megakaryocytes were of normal karyotype with no morphological abnormalities. Good's syndrome complicated by pure white cell aplasia (PWCA) was diagnosed. G-CSF was administered for eight days, but produced no response. Ten days after starting cyclosporine, neutrophils recovered and cyclosporine was tapered off. On admission, pharyngitis and enteritis were observed, which resolved with antibiotics, antifungal agents, G-CSF, and immunoglobulin supplementation. The anterior mediastinal mass was removed, and was diagnosed as thymoma type A. PWCA is rarely observed in Good's syndrome, and recurrence of PWCA after thymectomy poses a challenge in its treatment.

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