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Immunologic Research
Published in: Immunologic Research 1-3/2009

01-07-2009

Single-center analysis of long-term outcome after hematopoietic cell transplantation in children with congenital severe T cell immunodeficiency

Authors: Evelina Mazzolari, Donatella de Martiis, Concetta Forino, Arnalda Lanfranchi, Silvia Giliani, Roberto Marzollo, Paolo Airò, Luisa Imberti, Fulvio Porta, Luigi D. Notarangelo

Published in: Immunologic Research | Issue 1-3/2009

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Abstract

We review clinical outcome and immune reconstitution in a consecutive series of 74 infants with severe T cell immunodeficiency who received hematopoietic cell transplantation (HCT) from January 1991 to May 2003. Fifty-three patients (71.6%) are alive. Results were significantly better for recipients of HCT from HLA-matched related donors (100% survival) and unrelated donors (86.4%) than from mismatched related donors (51.6%). A detailed analysis of immune reconstitution and clinical status was performed in 49 surviving patients, most of which have attained robust T and B cell reconstitution and are in very good clinical conditions. No cases of late deaths or of chronic graft-versus-host disease (GvHD) have been observed. However, infections and autoimmunity at >1 year after HCT have been observed in a significant number of patients. Persistence of a low number of circulating naive T cells and long-term requirement for intravenous immunoglobulin were associated with a higher incidence of clinical events.
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Metadata
Title
Single-center analysis of long-term outcome after hematopoietic cell transplantation in children with congenital severe T cell immunodeficiency
Authors
Evelina Mazzolari
Donatella de Martiis
Concetta Forino
Arnalda Lanfranchi
Silvia Giliani
Roberto Marzollo
Paolo Airò
Luisa Imberti
Fulvio Porta
Luigi D. Notarangelo
Publication date
01-07-2009
Publisher
Humana Press Inc
Published in
Immunologic Research / Issue 1-3/2009
Print ISSN: 0257-277X
Electronic ISSN: 1559-0755
DOI
https://doi.org/10.1007/s12026-008-8022-4

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