Hepatitis C virus-induced cryoglobulinemia.
In this review we discuss the clinical manifestations, pathogenesis, and treatment of hepatitis C virus (HCV)-related cryoglobulinemia. HCV is a major cause of liver-related morbidity and is increasingly recognized as an instigator of B-cell lymphoproliferative disorders such as mixed cryoglobulinemia and non-Hodgkin lymphoma. Cryoglobulinemia is characterized by the clonal expansion of rheumatoid factor-expressing B cells in the liver, lymph nodes, and peripheral blood, resulting in the presence of cryoglobulins in the circulation. Cryoglobulins are cold-insoluble immune complexes containing rheumatoid factor, polyclonal IgG, and HCV RNA that precipitate and deposit on vascular endothelium, causing vasculitis in organs such as the skin, kidneys, and peripheral nerves. A subset of patients develops a low-grade lymphoma composed of B cells that are immunophenotypically similar to the expanded B cells seen in cryoglobulinemia. HCV-related B-cell lymphoproliferative disorders likely comprise a spectrum of disease, ranging from asymptomatic clonal B-cell expansions to pathogenic cryoglobulinemia and lymphoma. It is unclear how B cells become dysregulated during the course of chronic HCV infection, and continued patient-centered research is necessary to elucidate the pathogenesis of HCV-related B-cell dysregulation.
Charles
Edgar D
ED
Center for the Study of Hepatitis C, The Rockefeller University, New York, New York 10065, USA. charlee@rockefeller.edu
Dustin
Lynn B
LB
D000998:Antiviral Agents / Q000627:therapeutic use; D001402:B-Lymphocytes / Q000187:drug effects / Q000276:immunology / Q000821:virology*; D003449:Cryoglobulinemia / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology / Q000628:therapy / Q000821:virology*; D006526:Hepatitis C / Q000150:complications* / Q000188:drug therapy / Q000453:epidemiology / Q000276:immunology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008206:Lymphatic Diseases / Q000175:diagnosis / Q000276:immunology / Q000628:therapy / Q000821:virology*; D010956:Plasmapheresis; D016896:Treatment Outcome
D000998:Antiviral Agents; D007166:Immunosuppressive Agents