Nishizaki Yukiko, Osada Yuki, Kanai-Sudo Hikari, Mizuki Taro, Tanosaki Sakae, Suzuki Ken
Department of Hematology, Fraternity Memorial Hospital.
Rinsho Ketsueki. 2025;66(2):106-110. doi: 10.11406/rinketsu.66.106.
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.
一名58岁男性因1周前开始发热入院。他的白细胞计数为600/µl,中性粒细胞为0%,红细胞和血小板计数在正常范围内。骨髓增生低下,原始粒细胞占5.8%,但早幼粒细胞分化的粒细胞数量明显减少,M/E比值为0.18。成红细胞和巨核细胞核型正常,无形态学异常。诊断为合并纯白细胞再生障碍性贫血(PWCA)的古德综合征。给予粒细胞集落刺激因子(G-CSF)治疗8天,但无反应。开始使用环孢素10天后,中性粒细胞恢复,环孢素逐渐减量。入院时观察到咽炎和肠炎,使用抗生素、抗真菌药、G-CSF和补充免疫球蛋白后症状缓解。切除前纵隔肿块,诊断为A型胸腺瘤。PWCA在古德综合征中很少见,胸腺切除术后PWCA复发对其治疗构成挑战。