一名接受血液透析的终末期肾功能衰竭患者继发于边缘区淋巴瘤的混合性冷球蛋白血症血管炎。

IF 1 Q4 UROLOGY & NEPHROLOGY
CEN Case Reports Pub Date : 2024-06-01 Epub Date: 2023-10-06 DOI:10.1007/s13730-023-00823-5
Craig Peter Coorey, Amirhossein Aarabi, Karthik Kumar
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引用次数: 0

摘要

低温球蛋白血症血管炎可表现为多种症状,关于血液透析患者中低温球蛋白炎血管炎的发生率和表现的数据有限。我们报告了一例63岁的男性病例,他有一系列皮疹、视力变化、腹痛、体重减轻、发烧和指部缺血的表现。这是在一个患有糖尿病和高血压继发终末期肾衰竭的先天性单肾的背景下进行的,该肾接受了近5年的血液透析。他最初经历了皮肤活检证实的白细胞碎屑血管炎皮疹,随后多次因不明原因的未分化腹痛和发烧住院。空肠活检显示肠血管炎。他的外周血流式细胞术和骨髓活检与边缘区淋巴瘤(惰性亚型,IgMκ克隆)一致。进一步的测试显示II型冷球蛋白血症由IgMκ单克隆带和多克隆IgG组成(冷冻比容5%)。诊断为冷球蛋白血症血管炎,并对其进行了甲基强的松龙和利妥昔单抗脉冲治疗。然而,在接受三剂利妥昔单抗治疗后,患者出现了假定的血管炎相关肺出血,为此他接受了五次血浆置换治疗。他的症状缓解,冷冻比容降至
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Mixed cryoglobulinaemia vasculitis secondary to marginal zone lymphoma in a patient with end-stage renal failure on haemodialysis.

Cryoglobulinaemia vasculitis can present with a variety of symptoms and there is limited data on the incidence and presentation of cryoglobulinaemia vasculitis in haemodialysis patients. We report a case of a 63-year-old male who had a series of presentations with rash, visual changes, abdominal pain, weight loss, fevers and digital ischaemia. This is on a background of a congenital single kidney with end-stage renal failure secondary to diabetes and hypertension, receiving haemodialysis for nearly 5 years. He initially experienced a leukocytoclastic vasculitis rash confirmed on skin biopsy, followed by multiple hospital presentations for undifferentiated abdominal pain and fever of unknown source. Jejunal biopsy revealed intestinal vasculitis. His peripheral blood flow cytometry and bone marrow biopsy were consistent with marginal zone lymphoma (indolent subtype, IgM kappa clone). Further testing revealed a type II cryoglobulinaemia consisting of an IgM kappa monoclonal band with polyclonal IgG (cryocrit 5%). A diagnosis of cryoglobulinaemia vasculitis was established and he was treated with pulsed methylprednisolone and rituximab therapy. However, after receiving three doses of rituximab the patient developed a presumed vasculitis-associated pulmonary haemorrhage for which he received treatment with five sessions of plasma exchange. His symptoms resolved and cryocrit reduced to < 1% after his final dose of rituximab. The clinical features of cryoglobulinaemia may be difficult to detect in chronic haemodialysis patients and vigilance is required.

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来源期刊
CEN Case Reports
CEN Case Reports UROLOGY & NEPHROLOGY-
CiteScore
1.90
自引率
0.00%
发文量
80
期刊介绍: Clinical and Experimental Nephrology (CEN) Case Reports is a peer-reviewed online-only journal, officially published biannually by the Japanese Society of Nephrology (JSN).  The journal publishes original case reports in nephrology and related areas.  The purpose of CEN Case Reports is to provide clinicians and researchers with a forum in which to disseminate their personal experience to a wide readership and to review interesting cases encountered by colleagues all over the world, from whom contributions are welcomed.
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