Clinico‐biological characteristics and treatment of type I monoclonal cryoglobulinaemia: a study of 64 cases

• Published in: British journal of haematology Vol. 168; no. 5; pp. 671 - 678
• Main Authors: Harel, Stephanie, Mohr, Melanie, Jahn, Isabelle, Aucouturier, Francoise, Galicier, Lionel, Asli, Bouchra, Malphettes, Marion, Szalat, Raphael, Brouet, Jean‐Claude, Lipsker, Dan, Fermand, Jean‐Paul
• Format: Journal Article
• Language: English
• Published: England 01.03.2015
• Subjects: Aged; Antibodies, Monoclonal, Murine-Derived - administration & dosage; Antibodies, Monoclonal, Murine-Derived - adverse effects; Antineoplastic Agents - administration & dosage; Antineoplastic Agents - adverse effects; cryoglobulinaemia; Cryoglobulinemia - blood; Cryoglobulinemia - drug therapy; Cryoglobulinemia - pathology; Female; Follow-Up Studies; Humans; Immunoglobulin G - blood; Ischemia - blood; Ischemia - drug therapy; Ischemia - pathology; Kidney - pathology; Kidney Diseases - blood; Kidney Diseases - drug therapy; Kidney Diseases - pathology; Male; monoclonal gammopathy; Paraproteinemias - blood; Paraproteinemias - drug therapy; Paraproteinemias - pathology; Retrospective Studies; Rituximab; Skin - blood supply; Skin - pathology; Skin Diseases - blood; Skin Diseases - drug therapy; Skin Diseases - pathology; Waldenstrom Macroglobulinemia - blood; Waldenstrom Macroglobulinemia - drug therapy; Waldenstrom Macroglobulinemia - pathology; cryoglobulinaemia; monoclonal gammopathy
• ISSN: 0007-1048; 1365-2141; 1365-2141
• DOI: 10.1111/bjh.13196

Summary This retrospective analysis was conducted in 64 patients diagnosed with type I cryoglobulinaemia (CG) followed at two French centres. Median follow‐up was 6·75 years. CG was IgG in 60% and IgM in 40% of all cases and was asymptomatic in 16 patients (25%). Cold‐triggered ischaemic skin manifestations were observed in 33 patients (51%). Neurological manifestations were observed in 15 patients and renal manifestations in 13. Most of the patients with necrotic purpura (14/16, P = 0·009) and renal manifestations (11/13, P = 0·057) had IgG CG. IgG CG was associated with monoclonal gammopathy of undetermined significance (MGUS), myeloma, chronic lymphocytic leukaemia and lymphoplasmocytic lymphoma in 18, 13, 5 and 2 patients, respectively. IgM CG was associated with MGUS and Waldenström macroglobulinaemia in 8 and 18 cases, respectively. One third of patients did not receive any specific treatment. Various treatments, including rituximab, were administered to 25/31 patients with IgG CG and 6/25 patients with IgM CG due to CG‐related symptoms. Rituximab was ineffective in all cases associated with a predominantly plasmacytic proliferation. To conclude, type I CG has specific clinico‐biological characteristics compared to type II CG. Furthermore, there are differences in terms of related manifestations between type I IgG and type I IgM CG.