混合型冷球蛋白血症综合征伴有非 HCV B 细胞淋巴细胞增生性疾病,表现为坏疽和周围神经病变。

Rhode Island medical journal (2013) Pub Date : 2024-09-01
Ayomide Sorunke, Christopher Pinto, Muhammad Danish Saleem, Faryal Tahir
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引用次数: 0

摘要

混合型低温球蛋白血症是一种罕见的疾病,以坏疽、虚弱和关节痛为特征,可累及不同器官。它通常与丙型肝炎、艾滋病毒和免疫紊乱有关。诊断的依据是临床特征和检测冷球蛋白的血清学实验室检查。我们的患者是一名 64 岁的女性,表现为虚弱、疲劳、手指和脚趾变色。体格检查显示上肢和下肢皮肤发生变化,并伴有干性坏疽。血清学检查显示她没有丙型肝炎,低温球蛋白试验阳性,类风湿因子阳性,单克隆 IgM-kappa,确诊为混合型低温球蛋白血症。她接受了静脉注射免疫球蛋白、糖皮质激素、多个周期的利妥昔单抗、环磷酰胺和血浆置换治疗。在经历了需要进行膝下截肢的病情加重和复发的重大事件后,本病例报告旨在提高临床医生的认识,将其视为老年人坏疽和周围神经病变的罕见病因。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Mixed Cryoglobulinemia Syndrome Associated with Non-HCV B-Cell Lymphoproliferative Disorder Presenting with Gangrene and Peripheral Neuropathy.

Mixed cryoglobulinemia is a rare disorder characterized by gangrene, weakness, and arthralgias with variable organ involvement. It is often associated with hepatitis C, HIV, and immunological disorders. Diagnosis is based on clinical features and laboratory testing with serology detecting cryoglobulins. Our patient, a 64-year-old female, presented with weakness, fatigue, and discoloration of her fingers and toes. Physical examination showed upper- and lower-extremity skin changes with dry gangrene. Serology showed a non-hepatitis C status, positive cryoglobulin test with a positive rheumatoid factor, and monoclonal IgM-kappa, confirming the diagnosis of mixed cryoglobulinemia. She was treated with intravenous immunoglobulins, glucocorticoids, multiple cycles of rituximab, cyclophosphamide, and plasma exchange. Following a significant event of exacerbation and relapse requiring a below-knee amputation, this case report aims to raise awareness among clinicians to consider this as a rare cause of gangrene and peripheral neuropathy in an elderly adult.

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