REVIEW

Cryoglobulins

C Ferri¹, A L Zignego², S A Pileri³

¹ Dipartimento di Medicina Interna, Università di Pisa, Pisa 56126, Italy
² Dipartimento di Medicina Interna, Università di Firenze, Firenze 50134, Italy
³ Servizio di Anatomia Patologica ed Ematopatologia, Università di Bologna, Bologna 40100, Italy

Correspondence to:
Correspondence to:
Professor C Ferri, Dipartimento Medicina Interna-Reumatologia, Via Roma 67, 56126 Pisa, Italy;
c.ferri{at}int.med.unipi.it

Serum cryoglobulins are found in a wide spectrum of disorders but are often transient and without clinical implications. Monoclonal cryoglobulins are usually associated with haematological disorders, whereas mixed cryoglobulins are found in many infectious and systemic disorders. So called essential mixed cryoglobulinaemia shows a striking association with hepatitis C virus (HCV) infection (> 90%). It is a systemic vasculitis (leucocytoclastic vasculitis) with cutaneous and multiple visceral organ involvement. Chronic HCV infection can lead to a constellation of autoimmune and neoplastic disorders. In this review, the aetiology, diagnosis, disease heterogeneity, and treatment of cryoglobulinaemia are discussed.

Keywords: cryoglobulins; mixed cryoglobulinaemia; vasculitis; hepatitis C virus; autoimmunity; lymphoma; cancer

Abbreviations: APAAP, alkaline phosphatase anti-alkaline phosphatase; B-CLL, B cell chronic lymphocytic leukaemia/small lymphocytic lymphoma; BCL, B cell non-Hodgkin's lymphoma; ELISA, enzyme linked immunosorbent assay; Ig, immunoglobulin; LAC, low antigen content; MC, mixed cryoglobulinaemia; HBV, hepatitis B virus; HCV, hepatitis C virus; Ic, immunocytoma; MLDUS, monotypic lymphoproliferative disorder of undetermined significance; PCR, polymerase chain reaction; RF, rheumatoid factor;

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