Evans syndrome

An autoimmune disease in which an individual’s antibodies attack their own red blood cells and platelets.

The two events may occur together or sequentially.

A combination of autoimmune hemolytic anemia and immunologic thrombocytopenic purpura.

Reported that between 10% and 23% of patients who have autoimmune hemolytic anemia, will also have thrombocytopenia and thus Evans syndrome.

Autoantibodies targeted at antigens on red cells and platelets are assumed to cause isolated episodes of hemolytic anemia and thrombocytopenia, respectively.

Seen in subset of patient’s with common variable immunodeficiency.

The diagnosis is made by confirming hemolytic anemia and idiopathic thrombocytopenic purpura, but also a positive direct antiglobulin test (DAT) and an absence of any known underlying etiology.

Antibodies may occur directed against neutrophils and lymphocytes.

Referred to as an immunopancytopenia.

Initial treatment is with glucocorticoid corticosteroids or intravenous immunoglobulin, a procedure that is also used in ITP cases.

In children, steroids result in response in approximately 80 percent of cases.

Relapses are common and immunosuppressive drugs such as cyclosporine, mycophenolate mofetil, vincristine and danazol are considered

Rituximab may be beneficial in acute and refractory cases,

Splenectomy is effective in some cases.

Allogeneic hematopoietic stem cell transplantation may be curative.

Reported mortality rate of 7%.

Associated with a risk of developing other autoimmune problems and hypogammaglobulinemia,

More than 50% of children with Evans syndrome have CD4-/CD8- T cell ratio which is a strong predictor for having autoimmune lymphoproliferative syndrome.

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