Assuntos
Infecções por Vírus Epstein-Barr , Herpesvirus Humano 4/imunologia , Falência Renal Crônica/cirurgia , Transplante de Rim , Transtornos Linfoproliferativos , Segunda Neoplasia Primária , Adolescente , Linfócitos B/imunologia , Infecções por Vírus Epstein-Barr/etiologia , Infecções por Vírus Epstein-Barr/imunologia , Infecções por Vírus Epstein-Barr/terapia , Feminino , Humanos , Falência Renal Crônica/imunologia , Transtornos Linfoproliferativos/etiologia , Transtornos Linfoproliferativos/imunologia , Transtornos Linfoproliferativos/terapia , Transtornos Linfoproliferativos/virologia , Segunda Neoplasia Primária/etiologia , Segunda Neoplasia Primária/imunologia , Segunda Neoplasia Primária/terapia , Segunda Neoplasia Primária/virologia , Linfócitos T/imunologiaRESUMO
We retrospectively analyzed the outcomes of hematopoietic stem cell transplantation in 7 patients with primary immunodeficiency diseases treated at the National University Hospital, Singapore, over the period from December 1996 to January 2010. The primary immunodeficiency diseases managed were X-linked hyperimmunoglobulin M syndrome (n = 3), severe combined immunodeficiency (n = 1), leukocyte adhesion deficiency type 1 (n = 1), chronic granulomatous disease (n = 1), and Wiskott-Aldrich syndrome (n = 1). The age of the patients ranged from 5 months to 17 years. Conditioning regimen depended on the type of immunodeficiency, whereas supportive treatment was tailored for differing pretransplant conditions. Eight stem cell transplantations were performed for 7 patients. Donors were HLA-matched sibling donors for 2 patients and unrelated donors for the rest. At the median follow-up of 8.6 years (range 2.2-15.0 years) as of December 2011, 6 patients were alive and cured of their primary diseases.