Cryoglobulinemia

Cryoglobulinemia may be asymptomatic or present acutely.

Mixed cryoglobulinemia (MC) is the most common type, and it is strongly associated with chronic hepatitis C virus (HCV) infection.

Clinical presentation includes a triad of purpura, weakness, and arthralgia. Multisystemic involvement may include the kidneys, liver, and nervous system.

Diagnosis is based on clinical and laboratory findings. Leukocytoclastic vasculitis is the histologic hallmark of MC.

Management of MC secondary to chronic HCV infection includes viral eradication strategies in addition to immunosuppression.

Cryoglobulinemia refers to the presence of cryoglobulins in the serum. Cryoglobulins are immunoglobulins that reversibly precipitate at temperatures below 98.6°F (37°C). Type I cryoglobulinemia is usually associated with lymphoproliferative disorders (e.g., Waldenstrom macroglobulinemia). Types II and III (MC) may be associated with infectious (most commonly hepatitis C virus [HCV] infection) or autoimmune disorders.[1]Dispenzieri A, Gorevic PD. Cryoglobulinemia. Hematol Oncol Clin North Am. 1999 Dec;13(6):1315-49. http://www.ncbi.nlm.nih.gov/pubmed/10626153?tool=bestpractice.com [2]Tedeschi A, Barate C, Minola E, et al. Cryoglobulinemia. Blood Rev. 2007 Jul;21(4):183-200. http://www.ncbi.nlm.nih.gov/pubmed/17289231?tool=bestpractice.com ​ The clinical presentation includes a triad of purpura, weakness, and arthralgia. Multisystemic involvement may occur, particularly affecting the kidneys, liver, and nervous system. Widespread small vessel vasculitis has been demonstrated on post mortem studies.[3]Gorevic P, Kassab H, Levo Y, et al. Mixed cryoglobulinemia: clinical aspects and long term follow-up in 40 patients. Am J Med. 1980 Aug;69(2):287-308. http://www.ncbi.nlm.nih.gov/pubmed/6996482?tool=bestpractice.com [4]Ferri C. Mixed cryoglobulinemia. Orphanet J Rare Dis. 2008 Sep 16;3:25. http://ojrd.biomedcentral.com/articles/10.1186/1750-1172-3-25 http://www.ncbi.nlm.nih.gov/pubmed/18796155?tool=bestpractice.com