Hematopoietic Stem Cell Transplantation for 30 Patients with Primary Immunodeficiency Diseases: 20 Years Experience of a Single Team
Bone Marrow Transplantation (2006) 37, 469–477 & 2006 Nature Publishing Group All rights reserved 0268-3369/06 $30.00 www.nature.com/bmt ORIGINAL ARTICLE Hematopoietic stem cell transplantation for 30 patients with primary immunodeficiency diseases: 20 years experience of a single team Y Tsuji1, K Imai1,2, M Kajiwara1,3, Y Aoki1, T Isoda1, D Tomizawa1, M Imai1, S Ito1, H Maeda1, Y Minegishi1, H Ohkawa1, JYata 1, N Sasaki4, K Kogawa2, M Nagasawa1, T Morio1, S Nonoyama2 and S Mizutani1 1Department of Pediatrics and Developmental Biology, Graduate School of Medicine, Tokyo Medical and Dental University, Tokyo, Japan; 2Department of Pediatrics, National Defense Medical College, Saitama, Japan; 3Department of Blood Transfusion, University Hospital Faculty of Medicine, Tokyo Medical and Dental University, Tokyo, Japan and 4Department of Pediatrics, Saitama Medical School, Saitama, Japan We retrospectively analyzed our results of 30 patients with Introduction three distinctive primary immunodeficiency diseases (PIDs) – severe combined immunodeficiency (SCID, n ¼ 11), Primary immunodeficiency diseases (PIDs) are often Wiskott–Aldrich syndrome (WAS, n ¼ 11) and X-linked accompanied with life-threatening infections. Hematopoie- hyper-immunoglobulin M (IgM) syndrome (XHIM, n ¼ 8) tic SCT (HSCT) can be a treatment of choice to cure most – who underwent hematopoietic SCT (HSCT) during the of the lethal forms of immunodeficiencies, including severe past 20 years. Until 1995, all donors were HLA- combined immunodeficiency (SCID), Wiskott–Aldrich haploidentical relatives with T-cell depletion (TCD) syndrome (WAS) and X-linked hyper immunoglobulin M (n ¼ 8). Since 1996, the donors have been HLA-matched (IgM) syndrome (XHIM). An HLA-matched related donor related donors (MRD) (n ¼ 8), unrelated BM (UR-BM) (MRD), the best hematopoietic stem cell source, may not (n ¼ 7) and unrelated cord blood (UR-CB) (n ¼ 7).
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