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Evans syndromE

is an autoimmune disease in which an individual's antibodies attack their own red blood cells and platelets .Both of these events may occur simultaneously or one follow on from the other. Its overall pathology resembles a combination of autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura.

PATHOPHYSIOLOGY
The etiology of Evans syndrome is unknown. Norcross reacting autoantibodies are directed against antigens specific to red cells, platelets, or neutrophils. Wang et al demonstrated decreased levels of serum immunoglobulin (Ig) G, IgM, and IgA in these patients. The cytopenias that occur with Evans syndrome may be related to Tcell abnormalities because decreased Thelper cells and increased T-suppressor cells were noted in these patients.

CLINICAL MANIFESTATION
Patients may present with AIHA or ITP either separately. Thrombocytopenia; petichiae,bruising, mucocutaneous bleeding Examination may reveal lymphadenopathy, hepatomegaly and/or splenomegaly

TREATMENT First Line Therapy; corticosteroids


and/or intravenous immunoglobulin.

Second Line Therapy; include immunosuppressive drugs, especially cyclosporine or mycophenolate mofetil; vincristine; danazol or a combination of these agents.

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