Central Diagnostic Laboratory, Maastricht University Medical Center, P. Debyelaan 25, 6229 HX Maastricht, The Netherlands.
Autoimmun Rev. 2014 Apr-May;13(4-5):359-62. doi: 10.1016/j.autrev.2014.01.027. Epub 2014 Jan 11.
Cryoglobulinemia refers to the presence of reversible precipitation of immunoglobulins in the blood upon exposure to reduced (body) temperature. In clinical practice, the detection and typing of cryoglobulins appear to be poorly standardized. A consensus protocol for detection and typing of cryoglobulins, as extracted from the literature, is presented. Items that require further standardization are discussed. Cryoglobulins may cause clinical symptoms due to either obstruction of the small blood vessels, in particular in the extremities that are more exposed to the cold, or vascular inflammation due to the deposition of immune complexes. Only for the latter situation, i.e. cryoglobulinemic vasculitis in the presence of mixed-type cryoglobulinemia, preliminary classification criteria have been defined. Besides the presence of cryoglobulins, at least two of three items have to be present. These items include a restricted number of self-reported clinical history, typical clinical manifestations, and specified laboratory findings. These classification criteria await further validation in an independent patient cohort.
冷球蛋白血症是指在体温降低时血液中免疫球蛋白可逆性沉淀。在临床实践中，冷球蛋白的检测和分型似乎缺乏标准化。本文从文献中提取出冷球蛋白检测和分型的共识方案，并讨论了需要进一步标准化的项目。冷球蛋白可因小血管阻塞引起临床症状，特别是在更易暴露于寒冷的四肢，或因免疫复合物沉积引起血管炎症。仅在后一种情况下，即混合性冷球蛋白血症存在冷球蛋白血症性血管炎时，才定义了初步的分类标准。除了冷球蛋白的存在外，还必须至少出现以下三项中的两项：限定数量的自我报告的临床病史、典型临床表现和特定的实验室发现。这些分类标准有待在独立的患者队列中进一步验证。
