| Literature DB >> 23773507 |
Betul Tavil1, Arzu Yazal Erdem, Fatih Azik, Pamir Isik, Ayse Metin, Suna Emir, Duygu Uckan, Bahattin Tunc.
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.Entities:
Keywords: Wiskott-Aldrich syndrome; allogenic stem cell transplantation; non-Hodgkin lymphoma; pediatrics
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Year: 2013 PMID: 23773507 DOI: 10.1111/petr.12114
Source DB: PubMed Journal: Pediatr Transplant ISSN: 1397-3142