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Stable mixed chimerism after hematopoietic stem cell transplantation in Wiskott–Aldrich syndrome
Author(s) -
Doğu Figen,
KurtuluşÜlküer Melahat,
Bilge Yaşar,
Bozdoğan Günseli,
Ülküer Üner,
Malhatun Erdinç,
İkincioğulları Aydan,
Babacan Emel
Publication year - 2006
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/j.1399-3046.2005.00458.x
Subject(s) - wiskott–aldrich syndrome , medicine , hematopoietic stem cell transplantation , immunology , transplantation , haematopoiesis , stem cell , primary immunodeficiency , sibling , transplantation chimera , autoimmunity , savior sibling , immunodeficiency , immune system , hematopoietic cell , biology , psychology , developmental psychology , genetics , gene , biochemistry
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 × 10 9 /L), he is currently alive and doing well at 36 months post‐transplant and is free of any bleeding episodes.