Matches in SemOpenAlex for { <https://semopenalex.org/work/W4313247622> ?p ?o ?g. }
- W4313247622 abstract "Cytotoxic T-lymphocyte antigen-4 (CTLA-4) haploinsufficiency is a T-cell hyperactivation disorder that can manifest with both immunodeficiency and immune dysregulation. Approximately one-third of patients may present mild symptoms and remain stable under supportive care. The remaining patients may develop severe multiorgan autoimmunity requiring lifelong immunosuppressive treatment. Hematopoietic stem cell transplantation (HSCT) is potentially curable for patients with treatment-resistant immune dysregulation. Nevertheless, little experience is reported regarding the management of complications post-HSCT. We present case 1 (CTLA-4 haploinsufficiency) and case 2 (CTLA-4 insufficiency-like phenotype) manifesting with severe autoimmunity including cytopenia and involvement of the central nervous system (CNS), lung, and gut and variable impairment of humoral responses. Both patients underwent HSCT for which the main complications were persistent mixed chimerism, infections, and immune-mediated complications [graft-versus-host disease (GVHD) and nodular lung disease]. Detailed management and outcomes of therapeutic interventions post-HSCT are discussed. Concretely, post-HSCT abatacept and human leukocyte antigen (HLA)-matched sibling donor lymphocyte infusions may be used to increase T-cell donor chimerism with the aim of correcting the immune phenotype of CTLA-4 haploinsufficiency." @default.
- W4313247622 created "2023-01-06" @default.
- W4313247622 creator A5005413552 @default.
- W4313247622 creator A5011027535 @default.
- W4313247622 creator A5013160245 @default.
- W4313247622 creator A5013333007 @default.
- W4313247622 creator A5016882501 @default.
- W4313247622 creator A5025376171 @default.
- W4313247622 creator A5026096826 @default.
- W4313247622 creator A5042038610 @default.
- W4313247622 creator A5045653265 @default.
- W4313247622 creator A5054424036 @default.
- W4313247622 creator A5060445710 @default.
- W4313247622 creator A5062676244 @default.
- W4313247622 creator A5068740431 @default.
- W4313247622 creator A5074184309 @default.
- W4313247622 creator A5077389546 @default.
- W4313247622 date "2022-12-27" @default.
- W4313247622 modified "2023-10-18" @default.
- W4313247622 title "Case report: Challenges in immune reconstitution following hematopoietic stem cell transplantation for CTLA-4 insufficiency-like primary immune regulatory disorders" @default.
- W4313247622 cites W1817487237 @default.
- W4313247622 cites W2022738484 @default.
- W4313247622 cites W2034609134 @default.
- W4313247622 cites W2036687894 @default.
- W4313247622 cites W2053045221 @default.
- W4313247622 cites W2081255498 @default.
- W4313247622 cites W2089968188 @default.
- W4313247622 cites W2100304556 @default.
- W4313247622 cites W2122153475 @default.
- W4313247622 cites W2160429342 @default.
- W4313247622 cites W2171687266 @default.
- W4313247622 cites W2275605930 @default.
- W4313247622 cites W2339524645 @default.
- W4313247622 cites W2461116874 @default.
- W4313247622 cites W2554703541 @default.
- W4313247622 cites W2557740095 @default.
- W4313247622 cites W2597974085 @default.
- W4313247622 cites W2612689943 @default.
- W4313247622 cites W2622275391 @default.
- W4313247622 cites W2622876879 @default.
- W4313247622 cites W2757475539 @default.
- W4313247622 cites W2757670554 @default.
- W4313247622 cites W2793414942 @default.
- W4313247622 cites W2794587620 @default.
- W4313247622 cites W2801254160 @default.
- W4313247622 cites W2889742723 @default.
- W4313247622 cites W2902264758 @default.
- W4313247622 cites W2914208637 @default.
- W4313247622 cites W2972071232 @default.
- W4313247622 cites W2977747936 @default.
- W4313247622 cites W2981550624 @default.
- W4313247622 cites W2984852229 @default.
- W4313247622 cites W2996944141 @default.
- W4313247622 cites W3003117167 @default.
- W4313247622 cites W3008985781 @default.
- W4313247622 cites W3011527048 @default.
- W4313247622 cites W3087725160 @default.
- W4313247622 cites W3100356782 @default.
- W4313247622 cites W3126748203 @default.
- W4313247622 cites W3156868896 @default.
- W4313247622 cites W3159531940 @default.
- W4313247622 cites W3165501767 @default.
- W4313247622 cites W3168973386 @default.
- W4313247622 cites W3176386452 @default.
- W4313247622 cites W3180087324 @default.
- W4313247622 cites W3211391601 @default.
- W4313247622 cites W4214835238 @default.
- W4313247622 cites W4284882533 @default.
- W4313247622 cites W4296982417 @default.
- W4313247622 cites W4298126879 @default.
- W4313247622 doi "https://doi.org/10.3389/fimmu.2022.1070068" @default.
- W4313247622 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/36636328" @default.
- W4313247622 hasPublicationYear "2022" @default.
- W4313247622 type Work @default.
- W4313247622 citedByCount "1" @default.
- W4313247622 crossrefType "journal-article" @default.
- W4313247622 hasAuthorship W4313247622A5005413552 @default.
- W4313247622 hasAuthorship W4313247622A5011027535 @default.
- W4313247622 hasAuthorship W4313247622A5013160245 @default.
- W4313247622 hasAuthorship W4313247622A5013333007 @default.
- W4313247622 hasAuthorship W4313247622A5016882501 @default.
- W4313247622 hasAuthorship W4313247622A5025376171 @default.
- W4313247622 hasAuthorship W4313247622A5026096826 @default.
- W4313247622 hasAuthorship W4313247622A5042038610 @default.
- W4313247622 hasAuthorship W4313247622A5045653265 @default.
- W4313247622 hasAuthorship W4313247622A5054424036 @default.
- W4313247622 hasAuthorship W4313247622A5060445710 @default.
- W4313247622 hasAuthorship W4313247622A5062676244 @default.
- W4313247622 hasAuthorship W4313247622A5068740431 @default.
- W4313247622 hasAuthorship W4313247622A5074184309 @default.
- W4313247622 hasAuthorship W4313247622A5077389546 @default.
- W4313247622 hasBestOaLocation W43132476221 @default.
- W4313247622 hasConcept C126322002 @default.
- W4313247622 hasConcept C203014093 @default.
- W4313247622 hasConcept C21705690 @default.
- W4313247622 hasConcept C2776090121 @default.
- W4313247622 hasConcept C2777408962 @default.
- W4313247622 hasConcept C2780130043 @default.
- W4313247622 hasConcept C2780194698 @default.
- W4313247622 hasConcept C2911091166 @default.