[古德氏综合征合并纯白细胞发育不全]。

Yukiko Nishizaki, Yuki Osada, Hikari Kanai-Sudo, Taro Mizuki, Sakae Tanosaki, Ken Suzuki
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引用次数: 0

摘要

一名58岁男性因一周前开始发烧而入住我科。白细胞计数600/µl,中性粒细胞0%,红细胞、血小板计数正常。骨髓发育不全,成髓细胞占5.8%,但早幼粒细胞分化的粒细胞数量明显减少,M/E比为0.18。红细胞和巨核细胞核型正常,无形态学异常。诊断为古德氏综合征合并纯白细胞发育不全(PWCA)。G-CSF给药8天,但没有产生反应。使用环孢素10天后,中性粒细胞恢复,环孢素逐渐减少。入院时观察到咽炎和肠炎,经抗生素、抗真菌药物、G-CSF和补充免疫球蛋白解决。前纵隔肿块被切除,诊断为a型胸腺瘤。PWCA在Good's综合征中很少观察到,胸腺切除术后PWCA复发给其治疗带来了挑战。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Good's syndrome complicated with pure white cell aplasia].

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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