Allogeneic stem cell transplantation for Evans syndrome.
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.
Oyama
Y
Y
Division of Immune Therapy and Autoimmune Diseases, Northwestern University Medical Center, Chicago, IL, USA.
Papadopoulos
E B
EB
Miranda
M
M
Traynor
A E
AE
Burt
R K
RK
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000744:Anemia, Hemolytic, Autoimmune / Q000188:drug therapy / Q000601:surgery / Q000628:therapy*; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000601:surgery / Q000628:therapy*; D003131:Combined Modality Therapy; D003613:Danazol / Q000627:therapeutic use; D006086:Graft vs Host Disease / Q000209:etiology; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use; D000069285:Infliximab; D008297:Male; D009173:Mycophenolic Acid / Q000031:analogs & derivatives* / Q000627:therapeutic use; D009894:Opportunistic Infections / Q000209:etiology; D011241:Prednisone / Q000627:therapeutic use; D011697:Purpura, Thrombotic Thrombocytopenic / Q000209:etiology; D012074:Remission Induction; D016879:Salvage Therapy; D013156:Splenectomy; D013577:Syndrome; D013921:Thrombocytopenia / Q000188:drug therapy / Q000601:surgery / Q000628:therapy*; D019172:Transplantation Conditioning / Q000009:adverse effects; D014184:Transplantation, Homologous; D014750:Vincristine / Q000627:therapeutic use
D000305:Adrenal Cortex Hormones; D000911:Antibodies, Monoclonal; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D014750:Vincristine; D000069285:Infliximab; D009173:Mycophenolic Acid; D003613:Danazol; D011241:Prednisone