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[在福尼尔坏疽情况下诊断出的骨髓增生异常综合征]

[Myelodysplastic syndrome diagnosed on the occasion of Fournier's gangrene].

作者信息

Adachi Masaaki, Mitsuhashi Kimiyoshi, Matsuda Hiroyuki, Watanabe Junko, Nakanishi Katsuya

机构信息

Hematology Division, JCHO Sapporo Hokushin Hospital.

出版信息

Rinsho Ketsueki. 2017;58(2):113-118. doi: 10.11406/rinketsu.58.113.

Abstract

Fournier's gangrene (FG) is a fulminant infective necrotizing fasciitis, which includes the genital, perineal, and perianal regions. A 77-year-old man had previously been diagnosed as having diabetes mellitus (DM) and was treated with pioglitazone (15 mg) and miglitol (150 mg). He developed sudden perineal discomfort, fever with painful penile, and scrotal edema, subsequently leading to urinary retention. According to physical examination and CT scan results for the swollen penis and scrotum, he was diagnosed with FG. FG was eventually controlled by extensive treatment with broad spectrum antibiotics and repeated surgical debridement including penectomy and scrotectomy. He showed persistent anemia and decreased neutrophils exhibiting hypogranulation. Bone marrow aspiration revealed hypercellularity with 9% myeloblasts, micromegakaryocytes, abnormal leukocyte granulation, and erythrocytic dyspoiesis, leading to a diagnosis of myelodysplastic syndrome (MDS) RAEB-1, and he was evaluated as high risk according to IPSS-R. After 4 courses of azacitidine treatment, he achieved HI-E and had no further recurrence of FG for more than 18 months. Although DM and alcohol misuse are common systemic comorbidities in patients with FG, MDS should be considered in elderly FG cases, even when DM complications are present.

摘要

福尼尔坏疽(FG)是一种暴发性感染性坏死性筋膜炎,累及生殖器、会阴和肛周区域。一名77岁男性此前被诊断患有糖尿病(DM),接受吡格列酮(15毫克)和米格列醇(150毫克)治疗。他突然出现会阴部不适、阴茎疼痛伴发热以及阴囊水肿,随后导致尿潴留。根据肿大阴茎和阴囊的体格检查及CT扫描结果,他被诊断为FG。FG最终通过广谱抗生素的广泛治疗以及包括阴茎切除和阴囊切除在内的反复手术清创得以控制。他持续存在贫血,中性粒细胞减少,表现为颗粒减少。骨髓穿刺显示细胞增多,有9%的原始粒细胞、微小巨核细胞、异常白细胞颗粒和红细胞发育异常,导致诊断为骨髓增生异常综合征(MDS)RAEB - 1,根据国际预后评分系统 - 修订版(IPSS - R)评估为高危。经过4个疗程的阿扎胞苷治疗后,他达到了血液学改善,FG超过18个月未再复发。尽管DM和酒精滥用是FG患者常见的全身性合并症,但即使存在DM并发症,老年FG病例也应考虑MDS。

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