Cryoglobulinemia

background

Cryoglobulinemia is a medical condition that is caused by abnormal immunoglobulins (blood proteins), called cryoglobulins. Cryoglobulin literally means "cold antibody in the blood." Cryoglobulins are either single or mixed immunoglobulins that clump together at temperatures lower than 37 degrees Celsius. This process is reversed when they are warmed.
Cryoglobulins are often associated with diseases such as, hepatitis, multiple myeloma (type of bone marrow disease), dermatomyositis and lymphoma.
Since cryoglobulinemia makes the blood thicker, symptoms can arise throughout the body. Cryoglobulinemia primarily causes blood clots, stroke and vasculitis (inflamed blood vessels). Vasculitis of the arteries can result in blocked arteries, which often leads to damage in the organs that receive blood from the affected blood vessels (like the skin or kidneys).
The prognosis of cryoglobulinemic patients depends on the presence or absence of underlying diseases. Lymphoproliferative disorders, hepatitis B or C and connective tissue diseases are often associated with cryoglobulinemia, and these diseases increase the risk of complications (like kidney damage) or fatality. Death is usually the result of a serious heart disease, infection or brain hemorrhage (bleeding in the brain).
Treatment depends on the type of cryoglobulin, presence of an underlying disease and severity of symptoms. Cryoglobulinemia is generally treated with a combination of medications that reduce inflammation and suppress the immune system. Patients are encouraged to avoid cold temperatures, which trigger symptoms.

Related Terms

Acrocyanosis, chronic hepatitis C, chronic liver disease, chronic vasculitis, cryoprecipitation, cryoproteinemia, essential cryoglobulinemia, hepatitis C virus, HCV, hyperviscosity, idiopathic cryoglobulinemia, glomerulonephritis, IgE, IgM, immunoglobulin, immunoglobulin G, immunoglobulin M, livedo reticularis, protein, purpura, Raynaud phenomenon, Raynaud's phenomenon, retinal hemorrhage, secondary cryoglobulinemia, Sjögren syndrome, Sjögren's syndrome, simple cryoglobulinemia, SLE, stroke, systemic lupus erythematosus, thrombosis, type I cryoglobulinemia, type II cryoglobulinemia, vasculitis, Waldenström macroglobulinemia, Waldenström's macroglobulinemia.

classifications

Type I: Type I cryoglobulinemia, or simple cryoglobulinemia, is the result of a monoclonal (single) immunoglobulin, usually immunoglobulin M (IgM). This form is usually associated with lymphoproliferative diseases such as, Waldenström macroglobulinemia, multiple myeloma or chronic lymphocytic leukemia.
Type I cryoglobulinemia may result in hyperviscosity (extremely thick blood) due to high levels of circulating monoclonal cryoglobulin. This can lead to physical obstruction of blood vessels.
Type II and III: Types II and III, also known as the mixed cryoglobulinemias, involve cryoglobulin proteins which are a mixture of various antibody types that form for unknown reasons. The cryoglobulins contain rheumatoid factors (RFs). The RFs form complexes with the fragment, crystallizable portion of polyclonal (derived from multiple cells) immunoglobulin G (IgG). If the RF is monoclonal (derived from a single ancestral cell) it is considered Type II, and if the RF is polyclonal (derived from multiple cells), it is considered Type III.
Mixed cryoglobulinemias, including essential mixed cryoglobulinemia, make up about 80% of all cryoglobulinemia cases. They are associated with chronic inflammatory conditions like systemic lupus erythematosus (SLE), Sjögren syndrome (autoimmune disorder in which immune cells attack and destroy the glands that produce tears and saliva) and viral infections (particularly Hepatitis C virus). In fact, more than 90% of cryoglobulinemic patients develop the condition as an immune response to chronic hepatitis C infection.