2007, Number 4
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Acta Med 2007; 5 (4)
Refractory Evans syndrome with response to anti-CD 20 antibodies. Presentation of a case and review of the literature
Macías GE, Hurtado MR, Vargas VP
Language: Spanish
References: 22
Page: 209-213
PDF size: 87.50 Kb.
ABSTRACT
Patient 23-years-old female with Fisher-Evans syndrome diagnosis. The patient received metilprednisolone without complete remission. Splenectomy was done achieving partial response with persistent hemolysis. Anti-CD20 was given at 1g and the patient remains in complete remission for 15 months. Evans syndrome was first described by Evans in 1951 and is characterized by hemolytic anemia with Coombs direct positive and the association to autoimmune thrombocytopenia. First line treatment in Evans syndrome are corticosteroids. Intravenous immunoglobulin, splenectomy, azathioprine, and cyclosporine as well available treatment options. Rituximab (375 mg/m2/dosis), a monoclonal antibody anti-CD20, have shown effectiveness in this group of patients (Evans syndrome), they achieve the complete remission 15 months after completion of therapy. The rare occurrence of this disease and the response to anti-CD20, merit publication.
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