Cryoglobulinemia or cryoglobulinaemia is a medical condition in which the blood contains large amounts of cryoglobulins – proteins (mostly immunoglobulins themselves) that become insoluble at reduced temperatures. This should be contrasted with cold agglutinins, which cause agglutination of red blood cells. Cryoglobulins typically precipitate at temperatures below normal body temperature (37°C) and will dissolve again if the blood is heated. The precipitated clump can block blood vessels and cause toes and fingers to become gangrenous. Cryoglobulinemia can be associated with various diseases such as multiple myeloma and hepatitis C infection. Cryoglobulins usually consist of IgM directed against the Fc region of IgG.
Mild or moderate forms of cryoglobulinemia can often be treated by taking steps to deal with the underlying cause. Mild cases can be treated by avoiding cold temperatures. Standard hepatitis treatments work for most people who have hepatitis C and mild or moderate disease. The condition can come back when treatment stops. Severe cryoglobulinemia involves vital organs or large areas of skin. It is treated with corticosteroids and other medicines that suppress the immune system. Rituximab is an effective drug and has fewer risks than other medicines. Cyclophosphamide is used in life-threatening conditions where rituximab is not working or available. A treatment called plasmaphereis is also used. In this his procedure, blood plasma is taken out of blood circulation and abnormal cryoglobulin antibody proteins are removed. The plasma is replaced by fluid, protein, or donated plasma.
Major research on disease
Cryoglobulinemia is a type of vasculitis that is caused by abnormal proteins (antibodies) in the blood called "cryoglobulins." At cold temperatures, these proteins become solid or gel-like, which can block blood vessels and cause a variety of health problems. Many people affected by this condition will not experience any unusual signs or symptoms. When present, symptoms vary but may include breathing problems; fatigue; glomerulonephritis; joint or muscle pain; purpura; Raynaud's phenomenon; skin death; and/or skin ulcers. In some cases, the exact underlying cause is unknown; however, cryoglobulinemia can be associated with a variety of conditions including certain types of infection; chronic inflammatory diseases (such as autoimmune disease); and/or cancers of the blood or immune system. Treatment varies based on the severity of the condition, the symptoms present in each person and the underlying cause
Of 368 patients, 95% were female. Mean age at analysis was 55 y/o (21-87) and 50 y/o (20-89) at diagnosis. The frequency of PN was 11.68% (43/368). Sensory PN was found in 63% (28/43), predominantly small fibers involvement in 41.8% (18/43), axonal PN in 20.9% (9/43) and ataxic in 2.3% (1/43). Somatosensory manifestations were found in 37% (16/43) with axonal involvement in 30.2% (13/43) and mononeuritis multiplex in 6.9% (3/43), none had autonomic PN. When comparing groups (43 vs 172 controls) patients with PN had a higher frequency of vasculitis (11.7% vs 1.7%; p=0.002), purpura (23.8% vs 4.7%, p=0.0001), renal tubular acidosis (7.6% vs 1.2%, p=0.020), leucopenia (30.7% vs. 12.1%, p=0.005), low C3 (48.5% vs. 10.3%, p=0.0001) and C4 (66.6% vs. 18.2%, p=0.0001), (+) Anti-Ro/SSA (85.3% vs. 66.6%, p=0.019), (+) RF (72.5% vs. 52.1%, p=0.022), cryoglobulinemia (42.1% vs. 10.9%, p=0.0001) and higher frequency of hypergammaglobulinemia (60.5% vs 44.6%, p=0.09), Raynaud's (27.5% vs 11.6%, p=0.051) and glomerulonephritis (4.6% vs 0.5%, p=0.018), although without statistical significance.