Evans' Syndrome is an autoimmune disease in which an individual's antibodies attack their own RBCs as well as their platelets, both of these events may occur simultaneously or one follow on from the other. Its overall pathology approximates to a combination of the two autoimmune induced conditions: autoimmune hemolytic anemia and immune thrombocytopenic purpura.
Autoimmune hemolytic anemia is a condition in which the red blood cells that normally carry oxygen and carbon dioxide are destroyed by an autoimmune process. Immune thrombocytopenic purpura is a condition in which the platelets in the blood are destroyed by an autoimmune process. Platelets are a component of blood that contribute to the formation of blood clots in the body to prevent bleeding.
It has been variously reported that between 10% and 23% of patients who have autoimmune haemolytic anaemia, will also have thrombocytopenia and thus Evans syndrome. The two features may occur together or sequentially.
The precise cause is not yet known, but the immunology does differ from patients with just ITP with decreased T4 (T-helper), increased T8 (T-suppressor) and a decreased T4:T8 ratio. Together with decreased serum levels of IgG, IgM, and IgA these findings suggest an unsuccessful autoimmune response to an unknown trigger.
The diagnosis is made upon blood tests to confirm not only haemolytic anaemia and immune thrombocytopenic purpura, but also a positive direct antiglobulin test (DAT) and an absence of any known underlying aetiology.
Other antibodies may occur directed against neutrophils and lymphocytes, and "immunopancytopenia" has been suggested as a better term for this syndrome.
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Evans syndrome. Madisons Foundation (28 October 2003). Retrieved on 2007-10-29.
acquired hemolytic anemia: Autoimmune (Warm), HUS, MAHA, PNH, PCH