biostudies-literatureHaddad ENCATS NIH HHSNIAID NIH HHSNHLBI NIH HHSNCI NIH HHSNINDS NIH HHS1737-1749https://www.ebi.ac.uk/biostudies/studies/S-EPMC6202916biostudies-literatureUnknown132(17)The Primary Immune Deficiency Treatment Consortium (PIDTC) performed a retrospective analysis of 662 patients with severe combined immunodeficiency (SCID) who received a hematopoietic cell transplantation (HCT) as first-line treatment between 1982 and 2012 in 33 North American institutions. Overall survival was higher after HCT from matched-sibling donors (MSDs). Among recipients of non-MSD HCT, multivariate analysis showed that the SCID genotype strongly influenced survival and immune reconstitution. Overall survival was similar for patients with <i>RAG</i>, <i>IL2RG</i>, or <i>JAK3</i> defects and was significantly better compared with patients with <i>ADA</i> or <i>DCLRE1C</i> mutations. Patients with <i>RAG</i> or <i>DCLRE1C</i> mutations had poorer immune reconstitution than other genBloodSCID genotype and 6-month posttransplant CD4 count predict survival and immune recovery.PMC6202916U10 HL069254P30 CA008748R13 AI094943U24 CA076518U54 AI082973U01 TR001263U54 NS064808U01 HL069294Griffith LMPai SYHaddad EBednarski JJLogan BRBuckley RHSkoda-Smith SAbdel-Azim HSeroogy CMSzabolcs PKang EMCavanaugh MEHaight AEShereck EO'Reilly RJMalech HLHanson ICDesantes KBTaylor CGillio APKohn DBProckop SEKeller MDHeimall JKletzel MTumlin AGNotarangelo LDPetrovic AConnelly JADecaluwe HSullivan KECowan MJParrott REMartinez CThakar MSJyonouchi SLeiding JWYu LCPulsipher MAMoore TBMurnane MBleesing JJHSmith ARGoldman FDFleisher TAPuck JMPorteus MHQuigg TCChaisson JChandra SSmall TNCraddock JAKapoor NCuvelier GDEShyr DCDavila Saldana BJYin ZKnutsen APVander Lugt MTShearer WTAquino VTeira PBurroughs LDvorak CCDavis JHfalseSCID genotype and 6-month posttransplant CD4 count predict survival and immune recovery.The Primary Immune Deficiency Treatment Consortium (PIDTC) performed a retrospective analysis of 662 patients with severe combined immunodeficiency (SCID) who received a hematopoietic cell transplantation (HCT) as first-line treatment between 1982 and 2012 in 33 North American institutions. Overall survival was higher after HCT from matched-sibling donors (MSDs). Among recipients of non-MSD HCT, multivariate analysis showed that the SCID genotype strongly influenced survival and immune reconstitution. Overall survival was similar for patients with <i>RAG</i>, <i>IL2RG</i>, or <i>JAK3</i> defects and was significantly better compared with patients with <i>ADA</i> or <i>DCLRE1C</i> mutations. Patients with <i>RAG</i> or <i>DCLRE1C</i> mutations had poorer immune reconstitution than other gen2018-01-01T00:00:00Z2018 Oct2026-04-15T15:33:21.144Z2019-11-05T08:04:11ZS-EPMC62029163015411410.1182/blood-2018-03-840702