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在无明确感染源的疑似脓毒症中发现混合性冷球蛋白血症。

Unveiling Mixed Cryoglobulinemia in Suspected Sepsis Without a Source.

作者信息

Luna-Ceron Eder, Kattamuri Lakshmi, Vidal Katherine, Aguirre-Vera Guillermo de Jesus, Lehker Angelica

机构信息

Internal Medicine, Texas Tech University Health Sciences Center El Paso, El Paso, USA.

Clinical Sciences, Escuela de Medicina y Ciencias de la Salud Tecnologico de Monterrey, Mexico, MEX.

出版信息

Cureus. 2024 Apr 5;16(4):e57684. doi: 10.7759/cureus.57684. eCollection 2024 Apr.

DOI:10.7759/cureus.57684
PMID:38707103
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11070222/
Abstract

Cryoglobulinemia is an uncommon condition characterized by the presence of cryoprecipitable immune complexes in circulation, leading to clinical symptoms like purpura, muscle weakness, and joint pain. Specifically, mixed cryoglobulinemia involves the formation of these complexes due to rheumatoid factors, mainly IgM, occasionally IgG or IgA. Previously, Hepatitis C (HCV) was a common cause of mixed cryoglobulinemia, as the chronic HCV infection triggered immune responses that resulted in cryoglobulin formation. However, the emergence of direct-acting antivirals (DAAs) for HCV treatment has shifted the landscape, with autoimmune and lymphoproliferative disorders becoming more prominent etiological factors for mixed cryoglobulinemia. This case report features a 67-year-old woman with a history of Hepatitis C-related cirrhosis. She presented at the emergency department with signs of septic shock and widespread joint pain, particularly in the knees, shoulders, and neck. Effective sepsis management was achieved using antibiotics, albumin infusion, and midodrine. Nonetheless, significant cervical and bilateral knee pain persisted. Further examination uncovered hypocomplementemia and positive results for rheumatoid factors (IgA, IgM, IgG) and cryoglobulin agglutination, confirming the diagnosis of mixed cryoglobulinemia. This case emphasizes the importance of considering mixed cryoglobulinemia in chronic Hepatitis C patients displaying fatigue and joint pain, even in the absence of the traditional clinical manifestations. Moreover, the case underscores the dual benefits of DAA treatment for Hepatitis C in individuals with mixed cryoglobulinemia by achieving viral eradication and alleviating cryoglobulinemia-related symptoms, thus preventing further organ damage.

摘要

冷球蛋白血症是一种罕见病症,其特征是循环中存在可冷沉淀的免疫复合物,可导致紫癜、肌肉无力和关节疼痛等临床症状。具体而言,混合型冷球蛋白血症是由于类风湿因子(主要是IgM,偶尔是IgG或IgA)导致这些复合物形成。以前,丙型肝炎病毒(HCV)是混合型冷球蛋白血症的常见病因,因为慢性HCV感染引发免疫反应,导致冷球蛋白形成。然而,用于HCV治疗的直接抗病毒药物(DAA)的出现改变了局面,自身免疫性和淋巴增殖性疾病成为混合型冷球蛋白血症更突出的病因。本病例报告的患者是一名67岁女性,有丙型肝炎相关肝硬化病史。她因感染性休克体征和广泛的关节疼痛(尤其是膝盖、肩膀和颈部)就诊于急诊科。通过使用抗生素、输注白蛋白和米多君,有效控制了败血症。尽管如此,颈部和双侧膝盖仍有明显疼痛。进一步检查发现补体减少,类风湿因子(IgA、IgM、IgG)和冷球蛋白凝集试验结果呈阳性,确诊为混合型冷球蛋白血症。该病例强调,即使没有传统临床表现,对于出现疲劳和关节疼痛的慢性丙型肝炎患者,也需考虑混合型冷球蛋白血症。此外,该病例强调了DAA治疗丙型肝炎对混合型冷球蛋白血症患者的双重益处,即实现病毒清除和缓解冷球蛋白血症相关症状,从而防止进一步的器官损伤。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4552/11070222/194c77d2523f/cureus-0016-00000057684-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4552/11070222/194c77d2523f/cureus-0016-00000057684-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4552/11070222/194c77d2523f/cureus-0016-00000057684-i01.jpg

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