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type 1 (monoclonal) cryoglobulinemia
Etiology:
1) multiple myeloma
2) Waldenstrom's macroglobulinemia
3) MGUS [2]
4) B-cell non-Hodgkin's lymphoma, chronic lymphocytic leukemia [2]
5) Sjogren's syndrome
6) not strongly associated with hepatitis C
Pathology:
1) aggregates of a single monoclonal immunoglobulin
2) type-1 cryoglobulins generally do NOT activate complement
3) symptoms are generally related to hyperviscosity
4) ischemic ulceration from occlusion of arterioles & venules by precipitated immune complexes
Clinical manifestations:
1) patients generally asymptomatic
2) headaches
3) visual disturbances
4) nosebleeds
5) Raynaud's phenomenon 40%
6) skin ulcerations
7) vasculitis is uncommon (less common than types 2 & 3)
- lower extremity palpable purpura
- peripheral neuropathy, mononeuritis multiplex
- immune complex glomerulonephritis
8) acrocyanosis 15%
9) digital ischemia, distal necrosis 40%
10) livedo reticularis
Laboratory:
1) cryoglobulins in serum/plasma
- trace amounts of cryoglobulins may be found in normal individuals
2) serum protein electrophoresis & immunofixation electrophoresis
- high concentrations of monoclonal IgM - 1-5 g/dL
- IgG or IgA possible as well [2]
3) rheumatoid factor (RF) is negative
4) serum complement C3 in serum is generally low
4) serum complement C4 in serum is generally low
Management:
1) therapy directed at underlying disease
2) hyperviscosity responds to plasmapheresis
Related
cryoglobulin
General
cryoglobulinemia (cryoglobulinemic vasculitis)
References
- Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed)
Lippincott-Raven, Philadelphia, 1998, pg 849
- Medical Knowledge Self Assessment Program (MKSAP) 11, 17, 18, 19.
American College of Physicians, Philadelphia 1998, 2015, 2018, 2022.
- Brahn E, Scoville CD
Biochemical markers of disease activity.
Baillieres Clin Rheumatol 1988 2:153
PMID: 2458192