Long-Term Survival After Hematopoietic Stem Cell Transplantation for Complete STAT1 Deficiency.
Identifieur interne : 000440 ( Main/Merge ); précédent : 000439; suivant : 000441Long-Term Survival After Hematopoietic Stem Cell Transplantation for Complete STAT1 Deficiency.
Auteurs : Samuele Naviglio [Italie] ; Elena Soncini [Italie] ; Donatella Vairo [Italie] ; Arnalda Lanfranchi [Italie] ; Raffaele Badolato [Italie] ; Fulvio Porta [Italie]Source :
- Journal of clinical immunology [ 1573-2592 ] ; 2017.
Abstract
Complete signal transducer and activator of transcription 1 (STAT1) deficiency is a rare autosomal recessive condition characterized by impairment of intracellular signaling from both type I and type II interferons (IFN). Affected patients are prone to early severe mycobacterial and viral infections, which usually result in death before 18 months of age. We previously reported a patient affected by complete STAT1 deficiency who underwent hematopoietic stem cell transplantation (HSCT). Here, we describe the transplantation procedures and long-term outcomes.
DOI: 10.1007/s10875-017-0430-6
PubMed: 28815344
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pubmed:28815344Le document en format XML
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<front><div type="abstract" xml:lang="en">Complete signal transducer and activator of transcription 1 (STAT1) deficiency is a rare autosomal recessive condition characterized by impairment of intracellular signaling from both type I and type II interferons (IFN). Affected patients are prone to early severe mycobacterial and viral infections, which usually result in death before 18 months of age. We previously reported a patient affected by complete STAT1 deficiency who underwent hematopoietic stem cell transplantation (HSCT). Here, we describe the transplantation procedures and long-term outcomes.</div>
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