Cold agglutination syndrome

Cold agglutination syndrome is a rare autoimmune disorder characterized by the presence of high concentrations of circulating antibodies, known as cold agglutinins, that can agglutinate or clump together red blood cells at low temperatures. This syndrome is often associated with underlying conditions such as infections, lymphoproliferative disorders, and autoimmune diseases.

Etiology

Cold agglutination syndrome can be either primary (idiopathic) or secondary. Primary cold agglutination syndrome occurs without any identifiable cause. Secondary cold agglutination syndrome is associated with underlying conditions such as Mycoplasma pneumoniae infection, infectious mononucleosis, cytomegalovirus infection, HIV infection, lymphoma, multiple myeloma, and systemic lupus erythematosus.

Pathophysiology

In cold agglutination syndrome, the cold agglutinins, which are usually IgM antibodies, bind to the I antigen on the surface of red blood cells at low temperatures. This binding leads to agglutination of red blood cells, causing them to clump together. When the body temperature rises, the antibodies detach from the red blood cells, which can then circulate normally. However, if the agglutinated red blood cells pass through the liver or spleen, they may be recognized as abnormal and destroyed, leading to hemolytic anemia.

Clinical Manifestations

The symptoms of cold agglutination syndrome are often triggered by exposure to cold temperatures. These may include Raynaud's phenomenon, acrocyanosis (bluish discoloration of the extremities), and livedo reticularis (a mottled skin pattern). In severe cases, hemolytic anemia can occur, leading to symptoms such as fatigue, pallor, shortness of breath, and jaundice.

Diagnosis

Diagnosis of cold agglutination syndrome is based on the presence of high levels of cold agglutinins in the blood, detected by a cold agglutinin test. Other tests may include a complete blood count, liver function tests, and direct antiglobulin test.

Treatment

Treatment of cold agglutination syndrome is primarily aimed at managing the underlying condition, if any. Patients are advised to avoid exposure to cold temperatures. In severe cases, treatments such as plasmapheresis, immunosuppressive therapy, and rituximab may be used.

NIH genetic and rare disease info

• NIH info on Cold agglutination syndrome

Cold agglutination syndrome is a rare disease.

Cold agglutination syndrome Resources
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