Cryoglobulinemia classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Feham Tariq, MD [2]

Overview

Cryoglobulinemia is classically grouped into three types according to the Brouet classification. These are type 1, type 2 and type 3.

Classification

Cryoglobulinemia is classically grouped into three types according to the Brouet classification.[1]

Types of cryoglobulinemia
Associated diseases Type 1 Type 2 Type 3
  • Lymphoproliferative or plasma proliferative disorders(LPD)
  • Monoclonal gammopathy of undetermined significance (MGUS)
  • Hepatitis C virus
  • Connective tissue disease
  • Idiopathic
  • LPD
  • Hepatitis C virus
  • Connective tissue disease
  • Idiopathic
  • Other infections

Type I

  • It can lead to a glomerulopathy that is distinct from light chain disease in amyloidosis.

Type II

  • Type II is essential mixed cryoglobulinemia and the cryoglobulins are a polyclonal IgG and a momoclonal IgM rheumatoid factor directed against IgG.
  • Epstein-Barr Virus (EBV), HIV and Hepatitis B have been implicated but the majority is due to Hepatitis C (HCV).

Type III

  • Type III is also a mixed cryoglobulinemia (MC) where both the IgG and IgM are polyclonal.
  • It is seen in various autoimmune disorders and lymphoreticular disease as well as hepatitis C in almost 50%.

References

  1. Brouet JC, Clauvel JP, Danon F, Klein M, Seligmann M (1974). "Biologic and clinical significance of cryoglobulins. A report of 86 cases". Am. J. Med. 57 (5): 775–88. PMID 4216269.
  2. Ferri C, Zignego AL, Pileri SA (2002). "Cryoglobulins". J. Clin. Pathol. 55 (1): 4–13. PMID 11825916.


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