title: Successful unrelated cord blood transplantation in a 7-year-old boy with Evans syndrome refractory to immunosuppression and double autologous stem cell transplantation.
abstract: Evans syndrome is an autoimmunopathy characterized by thrombocytopenia and autoimmune hemolytic anemia with poor response to immunosuppression. A 2-yr-old boy with Evans syndrome showed only short-lasting responses to immunosuppressive treatment including double autologous peripheral stem cell transplantation (SCT). Intracranial bleeding required emergency splenectomy and external ventricular drainage. Unrelated umbilical cord blood was given following conditioning with busulfan, thiotepa, etoposide and antithymocyte globulin. One year after SCT the patient shows stable blood counts without immunosuppression. This is the first child reported with Evans syndrome successfully treated by means of unrelated cord blood transplantation.
authors.affiliation: Division of Pediatric Hematology/Oncology, Department of Pediatrics and Adolescent Medicine, Medical University of Graz, Graz, Austria.
authors.forename: Christian
authors.initials: C
authors.lastname: Urban
authors.forename: Herwig
authors.initials: H
authors.lastname: Lackner
authors.forename: Petra
authors.initials: P
authors.lastname: Sovinz
authors.forename: Martin
authors.initials: M
authors.lastname: Benesch
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Schwinger
authors.forename: Hans Jürgen
authors.initials: HJ
authors.lastname: Dornbusch
authors.forename: Andrea
authors.initials: A
authors.lastname: Moser
mesh_terms: D000305:Adrenal Cortex Hormones / Q000009:adverse effects / Q000627:therapeutic use; D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications / Q000188:drug therapy / Q000601:surgery*; D002543:Cerebral Hemorrhage / Q000209:etiology / Q000473:pathology; D002675:Child, Preschool; D036101:Cord Blood Stem Cell Transplantation; D003613:Danazol / Q000627:therapeutic use; D004351:Drug Resistance; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D036102:Peripheral Blood Stem Cell Transplantation; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000150:complications / Q000188:drug therapy / Q000601:surgery*; D012074:Remission Induction; D013156:Splenectomy; D019172:Transplantation Conditioning; D014182:Transplantation, Autologous; D014184:Transplantation, Homologous; D014750:Vincristine / Q000627:therapeutic use
chemical_list: D000305:Adrenal Cortex Hormones; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D014750:Vincristine; D003613:Danazol