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Classification and clinico-pathological characteristics of different cryoglobulinemias. |
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| Composition |
Pathological findings |
Clinical associations |
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| Type I cryoglobulinemia |
monoclonal Ig, mainly IgG, or IgM, or IgA self-aggregation through Fc fragment of Ig |
tissue histological alterations of underlying disorder |
-lymphoproliferative disorders: MM, WM, CLL, B-cell NHL |
| Type II mixed cryoglobulinemia |
monoclonal IgM (or IgG, or IgA) with RF activity (often cross-idiotype WA-mRF) and polyclonal Ig (mainly IgG) |
-leukocytoclastic vasculitis -B-lymphocyte expansion with tissue infiltrates |
-infections (mainly HCV) -autoimmune/lymphoproliferative disorders -rarely 'essential' |
| Type II–III mixed cryoglobulinemia |
oligoclonal IgM RF or mixture of poly/monoclonal IgM (often cross-idiotype WA-mRF) |
-leukocytoclastic vasculitis -B-lymphocyte expansion with tissue infiltrates |
-infections (mainly HCV) -autoimmune/lymphoproliferative disorders -rarely 'essential' |
| Type III mixed cryoglobulinemia |
polyclonal mixed Ig (all isotypes) with RF activity of one polyclonal component (usually IgM) |
-leukocytoclastic vasculitis -B-lymphocyte expansion with tissue infiltrates |
-infections (mainly HCV) -more often autoimmune disorders -rarely 'essential' |
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lymphoproliferative disorders: MM (multiple myeloma), WM (Waldenstrom's macroglobulinemia), chronic lymphocytic leukemia), B-cell non-Hodgkin's lymphoma; Ig: immunoglobulin; RF: rheumatoid factor; HCV: hepatitis C virus | |||
Ferri Orphanet Journal of Rare Diseases 2008 3:25 doi:10.1186/1750-1172-3-25 |
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