title: Successful allogeneic hemopoietic stem cell transplantation in a case of Wiskott-Aldrich syndrome and non-Hodgkin lymphoma.
abstract: WAS is a severe X-linked recessive disorder characterized by microthrombocytopenia, eczema, and immunodeficiency. A six-yr-old boy with WAS diagnosed as B-cell NHL (Stage III) localized in the liver who underwent successful HSCT from HLA-one antigen mismatch sibling donor has been presented here. His conditioning regimen included ATG, busulfan, and fludarabine. He received 2.3 × 10(6) /kg CD 34+ stem cells and 11 × 10(8) /kg nucleated cells at day 0. Neutrophil engraftment was achieved at day +14 and platelet engraftment at day +20. He has been in CR for more than two yr after transplantation. Thus, HSCT is an effective treatment for children with WAS even after development of lymphoma.
authors.affiliation: Department of Pediatric Hematology, Ankara Children's Hematology and Oncology Hospital, Diskapi, Ankara, Turkey. betultavil@yahoo.com
authors.forename: Betul
authors.initials: B
authors.lastname: Tavil
authors.forename: Arzu Yazal
authors.initials: AY
authors.lastname: Erdem
authors.forename: Fatih
authors.initials: F
authors.lastname: Azik
authors.forename: Pamir
authors.initials: P
authors.lastname: Isik
authors.forename: Ayse
authors.initials: A
authors.lastname: Metin
authors.forename: Suna
authors.initials: S
authors.lastname: Emir
authors.forename: Duygu
authors.initials: D
authors.lastname: Uckan
authors.forename: Bahattin
authors.initials: B
authors.lastname: Tunc
mesh_terms: D000961:Antilymphocyte Serum / Q000008:administration & dosage; D001792:Blood Platelets / Q000378:metabolism; D002066:Busulfan / Q000008:administration & dosage; D002648:Child; D006680:HLA Antigens / Q000737:chemistry; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods*; D006650:Histocompatibility Testing; D006801:Humans; D008113:Liver Neoplasms / Q000150:complications / Q000628:therapy; D008228:Lymphoma, Non-Hodgkin / Q000150:complications / Q000628:therapy*; D008297:Male; D009504:Neutrophils / Q000378:metabolism; D012074:Remission Induction; D035781:Siblings; D019172:Transplantation Conditioning / Q000379:methods; D016896:Treatment Outcome; D014740:Vidarabine / Q000008:administration & dosage / Q000031:analogs & derivatives; D014923:Wiskott-Aldrich Syndrome / Q000150:complications / Q000628:therapy*
chemical_list: D000961:Antilymphocyte Serum; D006680:HLA Antigens; D014740:Vidarabine; D002066:Busulfan; C024352:fludarabine
keywords: Wiskott-Aldrich syndrome; allogenic stem cell transplantation; non-Hodgkin lymphoma; pediatrics