Abstract: |
Evans syndrome is a rare disorder characterized by combined autoimmune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA). Standard treatments consist of transfusions, corticosteroids, splenectomy, IVIG, anabolic steroids, vincristine, alkylating agents, or cyclosporine. In a patient with refractory disease, an allogeneic hematopoietic stem cell transplant (HSCT) resulted in complete clinical and serologic remission for more than 30 months. Allogeneic HSCT may be the only current curative therapy for Evans syndrome but may also be complicated by significant toxicities. |
Keywords: |
adult; transplantation, homologous; danazol; prednisone; allogeneic stem cell transplantation; splenectomy; case report; salvage therapy; combined modality therapy; infection; thrombocytopenia; alkylating agent; cyclophosphamide; immunoglobulin; steroid; vincristine; plasmapheresis; hematopoietic stem cell transplantation; antibodies, monoclonal; syndrome; blood transfusion; graft versus host reaction; transplantation conditioning; remission; remission induction; azathioprine; autoimmune hemolytic anemia; corticosteroid; autoimmune diseases; autoimmune disease; tacrolimus; graft vs host disease; mycophenolic acid 2 morpholinoethyl ester; azithromycin; thrombotic thrombocytopenic purpura; serology; ganciclovir; pentamidine; cyclosporin; immunosuppressive agents; opportunistic infections; thymocyte antibody; immunoglobulins, intravenous; flucytosine; anabolic agent; viremia; amphotericin b lipid complex; adrenal cortex hormones; infliximab; beclometasone; interleukin 2 receptor antibody; mycophenolic acid; aerosol; purpura, thrombotic thrombocytopenic; fungemia; humans; human; male; priority journal; article; evans syndrome; anemia, hemolytic, autoimmune
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