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Its overall pathology resembles a combination of autoimmune hemolytic anemia and idiopathic 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. Idiopathic thrombocytopenic purpura is a condition in which platelets 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.
The syndrome was first described in 1951 by R. S. Evans and colleagues.
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Signs and symptoms
It has been variously reported that between 10% and 23% of patients who have autoimmune hemolytic anemia, will also have thrombocytopenia and thus Evans syndrome. The two features may occur together or sequentially.
Although Evans syndrome seems to be a disorder of immune regulation, the exact pathophysiology is unknown. Autoantibodies targeted at different antigenic determinants on red cells and platelets are assumed to cause isolated episodes of hemolytic anemia and thrombocytopenia, respectively.
The diagnosis is made upon blood tests to confirm not only hemolytic anemia and idiopathic thrombocytopenic purpura, but also a positive direct antiglobulin test (DAT) and an absence of any known underlying etiology.
Other antibodies may occur directed against neutrophils and lymphocytes, and "immunopancytopenia" has been suggested as a better term for this syndrome.
^ abcEvans RS, Takahashi K, Duane RT, Payne R, Liu C (1951). "Primary thrombocytopenic purpura and acquired hemolytic anemia; evidence for a common etiology". A.M.AARRAYrchives of internal medicine87 (1): 48–65. PMID14782741.
^Pegels JG, Helmerhorst FM, van Leeuwen EF, van de Plas-van Dalen C, Engelfriet CP, von dem Borne AE (1982). "The Evans syndrome: characterization of the responsible autoantibodies". Br. J. Haematol.51 (3): 445–50. doi:10.1111/j.1365-2141.1982.tb02801.x. PMID7104228.
^Liu H, Shao Z, Jing L (2001). "[The effectiveness of cyclosporin A in the treatment of autoimmune hemolytic anemia and Evans syndrome]". Zhonghua Xue Ye Xue Za Zhi (in Chinese) 22 (11): 581–3. PMID11855146.
^Yokoyama K, Kojima M, Komatsumoto S et al. (1992). "[Thrombotic thrombocytopenic purpura achieving complete remission by slow infusion of vincristine]". Rinsho Ketsueki (in Japanese) 33 (8): 1084–9. PMID1404865.
^Koike M, Ishiyama T, Saito K et al. (1993). "[Effective danazol therapy for a patient with Evans syndrome]". Rinsho Ketsueki (in Japanese) 34 (2): 143–6. PMID8492411.
^Hamidah A, Thambidorai CR, Jamal R (2005). "Prolonged remission after splenectomy for refractory Evans syndrome--a case report and literature review". Southeast Asian J. Trop. Med. Public Health36 (3): 762–4. PMID16124452.