title: Clinico-biological characteristics and treatment of type I monoclonal cryoglobulinaemia: a study of 64 cases.
abstract: 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.
authors.affiliation: Département d'Immuno-Hématologie, Hôpital Saint-Louis, Paris, France.
authors.forename: Stephanie
authors.initials: S
authors.lastname: Harel
authors.forename: Melanie
authors.initials: M
authors.lastname: Mohr
authors.forename: Isabelle
authors.initials: I
authors.lastname: Jahn
authors.forename: Francoise
authors.initials: F
authors.lastname: Aucouturier
authors.forename: Lionel
authors.initials: L
authors.lastname: Galicier
authors.forename: Bouchra
authors.initials: B
authors.lastname: Asli
authors.forename: Marion
authors.initials: M
authors.lastname: Malphettes
authors.forename: Raphael
authors.initials: R
authors.lastname: Szalat
authors.forename: Jean-Claude
authors.initials: JC
authors.lastname: Brouet
authors.forename: Dan
authors.initials: D
authors.lastname: Lipsker
authors.forename: Jean-Paul
authors.initials: JP
authors.lastname: Fermand
mesh_terms: D000368:Aged; D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage* / Q000009:adverse effects; D000970:Antineoplastic Agents / Q000008:administration & dosage* / Q000009:adverse effects; D003449:Cryoglobulinemia / Q000097:blood / Q000188:drug therapy / Q000473:pathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D007511:Ischemia / Q000097:blood / Q000188:drug therapy / Q000473:pathology; D007668:Kidney / Q000473:pathology; D007674:Kidney Diseases / Q000097:blood / Q000188:drug therapy / Q000473:pathology; D008297:Male; D010265:Paraproteinemias / Q000097:blood / Q000188:drug therapy / Q000473:pathology; D012189:Retrospective Studies; D000069283:Rituximab; D012867:Skin / Q000098:blood supply / Q000473:pathology; D012871:Skin Diseases / Q000097:blood / Q000188:drug therapy / Q000473:pathology; D008258:Waldenstrom Macroglobulinemia / Q000097:blood / Q000188:drug therapy / Q000473:pathology
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D007074:Immunoglobulin G; D000069283:Rituximab
keywords: cryoglobulinaemia; monoclonal gammopathy