Dammacco F, Scarpioni L, Antonaci S, Bonomo L
Acta Haematol. 1978;59(4):215-22. doi: 10.1159/000207764.
Mixed-typed cryoproteins, consisting of IgG and IgM, were demonstrated in the sera of four sisters. While the IgG component was polyclonal in every instance, in two of them the IgM component was found to be monoclonal with type chi light chains. Clinical diagnoses included the purpura-weakness-arthralgias syndrome, posthepatitis cirrhosis, congestive heart failure and mitral stenosis. The cryocrit differed in the four sisters, ranging from 3 to 16%; in addition, rheumatoid factor activity was consistently associated with both washed cryoprecipitates and their isolated IgM components. Endomembraneous deposits of IgG and IgM were revealed by immunofluorescent studies of the renal biopsy specimen from one patient. A genetic abnormality, possibly of the autosomal recessive type, is suggested in this instance of familial cryoglobulinemia.
在四姐妹的血清中发现了由IgG和IgM组成的混合型冷球蛋白。虽然IgG成分在每种情况下都是多克隆的，但其中两例的IgM成分被发现是具有κ型轻链的单克隆成分。临床诊断包括紫癜-虚弱-关节痛综合征、肝炎后肝硬化、充血性心力衰竭和二尖瓣狭窄。四姐妹的冷球蛋白比容不同，范围为3%至16%；此外，类风湿因子活性始终与洗涤后的冷沉淀物及其分离出的IgM成分相关。对一名患者的肾活检标本进行免疫荧光研究，发现有IgG和IgM的内膜沉积。在这例家族性冷球蛋白血症中提示存在一种可能为常染色体隐性类型的基因异常。
