PDF(Pubmed)
Abstract：
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.
摘要：
冷球蛋白血症性血管炎可表现出多种症状，关于血液透析患者冷球蛋白血症性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，他出现了一系列皮疹，视觉变化,腹痛,减肥,发烧和数字缺血。这是在先天性单肾伴有糖尿病和高血压继发的终末期肾衰竭的背景下，接受血液透析近5年。他最初经历了皮肤活检证实的白细胞碎裂性血管炎皮疹，随后是多次医院就诊的未分化腹痛和不明原因发热。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符合边缘区淋巴瘤（惰性亚型，IgMκ克隆)。进一步的测试显示II型冷球蛋白血症，由具有多克隆IgG（冷冻比容5％）的IgMκ单克隆带组成。确定了冷球蛋白血症血管炎的诊断，并接受了脉冲甲基强的松龙和利妥昔单抗治疗。然而,在接受三剂利妥昔单抗治疗后,患者出现了推测的血管炎相关性肺出血,并接受了5次血浆置换治疗.在他最后一次服用利妥昔单抗后，他的症状得到缓解，冷冻比容降低至<1%。慢性血液透析患者可能难以发现冷球蛋白血症的临床特征，需要保持警惕。
