Damoiseaux Jan, Cohen Tervaert Jan Willem
Central Diagnostic Laboratory, Maastricht University Medical Center, P. Debyelaan 25, 6229 HX, Maastricht, The Netherlands,
Clin Rev Allergy Immunol. 2014 Dec;47(3):299-310. doi: 10.1007/s12016-013-8390-y.
Cryoglobulins are immunoglobulins that reversibly precipitate in the cold. They come in different flavours and, as such, are differentially associated with lymphoproliferative diseases (type I), or systemic autoimmune diseases, and/or infectious diseases (type II/III). The clinical manifestations of cryoglobulinaemia result from either hyper-viscosity or small vessel vasculitis. Hepatitis C virus (HCV) is a well-known factor in the aetiology of cryoglobulinaemia, but substantial geographical differences exist in the association between cryoglobulins and HCV. In the absence of any underlying disease, cryoglobulinaemia is referred to as 'idiopathic' or 'essential'. Detection of cryoglobulins in the laboratory is hampered by several pitfalls, in particular in the pre-analytical stage as well as in the quantification. In addition, our personal experience reveals that the detection of rheumatoid factor, most often present in high concentrations in patients with mixed cryoglobulinaemia, relies on the choice of the test system. Hence, interpretation of the laboratory results in relation to the clinical manifestations requires a partnership between the clinician and the laboratory specialist in order to make a correct diagnosis. Treatment options are primarily directed by identification of underlying diseases, i.e. infections or systemic autoimmune diseases. Idiopathic cryoglobulinaemia is treated with corticosteroids and immunosuppression, or B cell depleting anti-CD20 biologicals. In this overview, the recent literature on current laboratory and clinical practice of cryoglobulinaemia is discussed from a personal perspective.
冷球蛋白是在低温下可逆性沉淀的免疫球蛋白。它们有不同类型，因此与淋巴增殖性疾病（I型）、或系统性自身免疫性疾病、和/或感染性疾病（II/III型）有不同的关联。冷球蛋白血症的临床表现源于血液高黏滞度或小血管血管炎。丙型肝炎病毒（HCV）是冷球蛋白血症病因中一个众所周知的因素，但冷球蛋白与HCV之间的关联存在显著的地域差异。在没有任何潜在疾病的情况下，冷球蛋白血症被称为“特发性”或“原发性”。实验室中冷球蛋白的检测存在几个陷阱，特别是在分析前阶段以及定量方面。此外，我们的个人经验表明，类风湿因子的检测（在混合性冷球蛋白血症患者中通常浓度较高）取决于检测系统的选择。因此，结合临床表现对实验室结果进行解读需要临床医生和实验室专家之间的合作，以便做出正确的诊断。治疗方案主要取决于潜在疾病的识别，即感染或系统性自身免疫性疾病。特发性冷球蛋白血症用皮质类固醇和免疫抑制治疗，或使用耗竭B细胞的抗CD20生物制剂治疗。在本综述中，从个人角度讨论了有关冷球蛋白血症当前实验室和临床实践的最新文献。
