Subject(s)
Epstein-Barr Virus Infections , Herpesvirus 4, Human/immunology , Kidney Failure, Chronic/surgery , Kidney Transplantation , Lymphoproliferative Disorders , Neoplasms, Second Primary , Adolescent , B-Lymphocytes/immunology , Epstein-Barr Virus Infections/etiology , Epstein-Barr Virus Infections/immunology , Epstein-Barr Virus Infections/therapy , Female , Humans , Kidney Failure, Chronic/immunology , Lymphoproliferative Disorders/etiology , Lymphoproliferative Disorders/immunology , Lymphoproliferative Disorders/therapy , Lymphoproliferative Disorders/virology , Neoplasms, Second Primary/etiology , Neoplasms, Second Primary/immunology , Neoplasms, Second Primary/therapy , Neoplasms, Second Primary/virology , T-Lymphocytes/immunologyABSTRACT
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.
