Cryoglobulinemia

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The cryoglobulinemia belongs to the group of vasculitis (vasculitides) which do not ANCA are -assoziiert.

First describer

Wintrobe and Buell 1933 56-year-old woman with multiple myeloma and "kältepräzipitierenden serum globulins".

In 1947, Lerner and Watson described a patient with unusual purpura and high concentrations of "cold-precipitating serum globulins", which they called cryoglobulins and thus coined the term.

Pathogenesis

Cryoglobulins are antibodies ( immunoglobulins ) that become insoluble when cold and redissolve when heated. In addition, they can have the properties of rheumatoid factors or cold agglutinins . A distinction is made between monoclonal cryoglobulins and mixed cryoglobulins . The latter are immune complexes .

There are three different types of cryoglobulins:

  • Type 1 cryoglobulins : monoclonal immunoglobulin G (IgG) or immunoglobulin M (IgM), rarely other immunoglobulins. Monoclonal cryoglobulins.
  • Type 2 cryoglobulins : mostly monoclonal immunoglobulin M, which, like rheumatoid factors, binds to the Fc piece of IgG. Mixed cryoglobulins.
  • Type 3 cryoglobulins : polyclonal immunoglobulin M that forms immune complexes with another polyclonal immunoglobulin. Mixed cryoglobulins.

Chronic hepatitis C causes cryoglobulinemia in about 80 percent of cases . Other causes can be:

Symptoms

  • Skin symptoms:
  • Arthralgia (joint pain)
  • Myalgia (muscle pain)
  • Focus neuropathy
  • Liver / spleen enlargement in 60 percent of patients (especially in Waldenström's disease and plasmacytoma)
  • Lymph node disease
  • Glomerulonephritis with hematuria and proteinuria
  • neuropathy in 50 percent of patients
  • multiple organ attacks

diagnosis

The diagnosis is based on anamnesis , vascular biopsy and possible evidence of hepatitis C. The sedimentation rate is strongly accelerated at 37 ° C and normal at 4 ° C. The cryoglobulins can be detected by electrophoresis .

treatment

On the one hand, possible underlying diseases are treated, on the other hand, corticosteroids and various chemotherapeutic agents are used.

Individual evidence

  1. ^ Bulletin of the Johns Hopkins Hospital , 1933, 52, p. 156
  2. American Journal of Medical Sciences , 1947, 214, p. 410
  3. Brouet et al. In: Am J Med , 1974, 57, pp. 775-788.