Successful allogeneic hemopoietic stem cell transplantation in a case of Wiskott-Aldrich syndrome and non-Hodgkin lymphoma

Tavil B., Erdem A. Y., Azik F., Isik P., Metin A., Emir S., ...Daha Fazla

Pediatric Transplantation, cilt.17, sa.6, 2013 (SCI-Expanded) identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 17 Sayı: 6
  • Basım Tarihi: 2013
  • Doi Numarası: 10.1111/petr.12114
  • Dergi Adı: Pediatric Transplantation
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Anahtar Kelimeler: allogenic stem cell transplantation, non-Hodgkin lymphoma, pediatrics, Wiskott-Aldrich syndrome
  • Hacettepe Üniversitesi Adresli: Hayır

Özet

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 × 106/kg CD 34(+) stem cells and 11 × 108/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. © 2013 John Wiley & Sons A/S.