Cryoglobulinemia

The cryoglobulinemia belongs to the group of vasculitis (blood vessel inflammation ) that are not ANCA -associated.

Erstbeschreiber

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

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

Pathogenesis

Cryoglobulins are antibodies (immunoglobulins ) that are insoluble in cold and in heat go back into solution. Additionally, they may have the characteristics of rheumatoid factors or cold agglutinins. We distinguish between monoclonal cryoglobulins and mixed cryoglobulins. For the latter is immune complexes.

There are three different types of cryoglobulins ( Brouet et al, Am J Med 1974; 57:775-88. ):

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

In about 80 percent of cases of cryoglobulinemia is based on a chronic hepatitis C. Other causes can be:

  • Waldenstrom
  • Multiple Myeloma
  • Systemic lupus erythematosus
  • Sjogren's Syndrome
  • Polyarteritis nodosa
  • Rheumatoid arthritis
  • Sarcoidosis
  • Ulcerative colitis
  • Infections with: Epstein-Barr virus (EBV)
  • Toxoplasma gondii, the causative agent of toxoplasmosis
  • Treponema pallidum, the causative agent of syphilis
  • Borrelia
  • Cytomegalovirus ( CMV)

Symptoms

  • Skin symptoms: Called skin bleeding due to vascular wall damage acral, these skin manifestations are called " palpable purpura "
  • Raynaud's Disease
  • Acrocyanosis
  • Necrosis of acral

Diagnosis

The diagnosis is normally based on medical history, vascular biopsies and possible evidence of hepatitis C. The erythrocyte sedimentation rate is greatly accelerated at 37 ° C, at 4 ° C. The cryoglobulins can be detected by electrophoresis.

Treatment

For one possible reason diseases are treated for other corticosteroids and various chemotherapeutic agents are used.

Swell

  • Disease in hematology and oncology
Anti-neutrophil cytoplasmic antibody Epstein–Barr virus Glomerulonephritis
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