title: The cryoglobulinemic syndrome.
abstract: The clinical and immunochemical classification of 376 patients with cryoglobulinemia is presented. In 141 cases (37.5%) the syndrome was considered idiopathic. Among the secondary forms, chronic liver diseases and connective tissue diseases shared most frequently mixed cryoglobulins (18.8 and 15.4% of cases, respectively). Another common association was that with Waldenström's macroglobulinemia: cryoglobulins were found in 20% of these patients. In 5 patients (2 with the essential form and 3 with cryoglobulinemia secondary to Waldenström's disease) a Cl-inhibitor deficiency was discovered; the complement profile was characteristic of the acquired type and episodes of angioedema occurred. Studying the clinical course of 71 cases of essential mixed cryoglobulinemia followed-up for 4-20 years we concluded that the evolution of type II and type III cryoglobulinemias is different because that of type II seems to have a more severe course and may differentiate in a lymphoproliferative disorder; the immunochemical type of cryoglobulins seems therefore to have a prognostic significance. Because of the considerable overlap in the distribution of immunochemical types among the clinical subsets, a mixed classification (both biochemical and clinical) is proposed.
mesh_terms: D000328:Adult; D000368:Aged; D003449:Cryoglobulinemia / Q000145:classification / Q000150:complications / Q000276:immunology / Q000503:physiopathology*; D003450:Cryoglobulins / Q000276:immunology / Q000378:metabolism; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007105:Immune Complex Diseases / Q000097:blood; D007674:Kidney Diseases / Q000209:etiology; D008232:Lymphoproliferative Disorders / Q000097:blood; D008297:Male; D008875:Middle Aged; D010266:Paraproteins / Q000378:metabolism; D008258:Waldenstrom Macroglobulinemia / Q000503:physiopathology
chemical_list: D003450:Cryoglobulins; D010266:Paraproteins