Stable mixed chimerism after hematopoietic stem cell transplantation in Wiskott-Aldrich syndrome

DOĞU E. F., Kurtulus-Ulkuer M., Bilge Y., Bozdogan G., Ulkuer U., Malhatun E., ...More

PEDIATRIC TRANSPLANTATION, vol.10, no.3, pp.395-399, 2006 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 10 Issue: 3
  • Publication Date: 2006
  • Doi Number: 10.1111/j.1399-3046.2005.00458.x
  • Journal Name: PEDIATRIC TRANSPLANTATION
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.395-399
  • Keywords: Wiskott-Aldrich syndrome, mixed chimerism, STR, BMT, BONE-MARROW-TRANSPLANTATION, SINGLE-CENTER, CHILDREN, IMPACT
  • Ankara University Affiliated: Yes

Abstract

Wiskott-Aldrich syndrome (WAS) is a primary immunodeficiency disease characterized by thrombocytopenia, eczema, impaired cellular and humoral immunity, and increased susceptibility to malignancy and autoimmunity. The only curative treatment for WAS is hematopoietic stem cell transplantation, especially in the presence of a matched sibling donor or matched unrelated donor. Here, we report the case of a 2.5-yr-old boy with WAS that resulted in mixed chimerism after having received bone marrow from his phenotypically identical grandfather. Although the patient has persistent thrombocytopenia (platelet counts 50-80 x 10(9)/L), he is currently alive and doing well at 36 months post-transplant and is free of any bleeding episodes.