Ravulizumab Can Effectively Treat Ischemic Enteritis Caused by Paroxysmal Nocturnal Hemoglobinuria.

IF 1.2 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL
Journal of Nippon Medical School Pub Date : 2024-11-06 Epub Date: 2023-08-08 DOI:10.1272/jnms.JNMS.2024_91-505
Atsushi Marumo, Haruka Okabe, Hisae Sugihara, Masanobu Eguchi
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引用次数: 0

Abstract

Ischemic colitis is a common disease with a good prognosis; however, complications can occur in the presence of a serious underlying disease. Herein, we present a case report in which characteristic findings on lower gastrointestinal endoscopy led to a diagnosis of paroxysmal nocturnal hemoglobinuria (PNH). An 82-year-old woman visited our hospital for chronic heart and renal failure. She had a history of breast cancer, myocardial infarction, and hemorrhoidal fistula and was initially hospitalized for ischemic colitis. Subsequent lower gastrointestinal endoscopy revealed extensive ulcerative lesions in the ascending, transverse, and descending colon. Histopathologically, small vessels exhibited multiple fibrin thrombus formations. Based on histopathological and endoscopic results, the presence of an underlying disease was suspected. Flow cytometric analysis showed that erythrocytes and granulocytes had 5.5 and 86.4% CD55- and CD59-negative cells, respectively. The patient was ultimately diagnosed with PNH and considered severely ill, given the ischemic colitis-induced abdominal pain and the need for red blood cell transfusions (4-6 units per month). Accordingly, the patient was administered ravulizumab. Ischemic enteritis did not relapse following ravulizumab administration, and transfusion dependence improved. If a patient with ischemic colitis presents atypical lower gastrointestinal endoscopic findings, it is important to explore the presence of an underlying disease.

雷珠单抗可有效治疗阵发性夜间血红蛋白尿引起的缺血性肠炎
缺血性结肠炎是一种常见疾病,预后良好;但如果存在严重的潜在疾病,则可能出现并发症。在本报告中,我们介绍了一例病例,该病例的下消化道内窥镜检查发现的特征性结果导致了阵发性夜间血红蛋白尿(PNH)的诊断。一位 82 岁的妇女因慢性心肾功能衰竭来我院就诊。她曾患乳腺癌、心肌梗塞和痔瘘,最初因缺血性结肠炎住院治疗。随后的下消化道内窥镜检查发现,升结肠、横结肠和降结肠有广泛的溃疡性病变。组织病理学检查显示,小血管有多处纤维蛋白血栓形成。根据组织病理学和内镜检查结果,怀疑患者存在潜在疾病。流式细胞分析显示,红细胞和粒细胞中 CD55 阴性细胞占 5.5%,CD59 阴性细胞占 86.4%。鉴于缺血性结肠炎引起的腹痛以及需要输注红细胞(每月 4-6 单位),患者最终被诊断为 PNH,并被认为病情严重。因此,患者接受了雷珠单抗治疗。使用雷珠单抗后,缺血性肠炎没有复发,输血依赖性也有所改善。如果缺血性结肠炎患者出现不典型的下消化道内窥镜检查结果,就必须探究是否存在潜在疾病。
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来源期刊
Journal of Nippon Medical School
Journal of Nippon Medical School MEDICINE, GENERAL & INTERNAL-
CiteScore
1.80
自引率
10.00%
发文量
118
期刊介绍: The international effort to understand, treat and control disease involve clinicians and researchers from many medical and biological science disciplines. The Journal of Nippon Medical School (JNMS) is the official journal of the Medical Association of Nippon Medical School and is dedicated to furthering international exchange of medical science experience and opinion. It provides an international forum for researchers in the fields of bascic and clinical medicine to introduce, discuss and exchange thier novel achievements in biomedical science and a platform for the worldwide dissemination and steering of biomedical knowledge for the benefit of human health and welfare. Properly reasoned discussions disciplined by appropriate references to existing bodies of knowledge or aimed at motivating the creation of such knowledge is the aim of the journal.
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