Journal article
Morbidity, Mortality, and Therapeutics in Combined Immunodeficiency: Data From the USIDNET Registry
The journal of allergy and clinical immunology in practice (Cambridge, MA), Vol.10(5), pp.1334-1341.e6
05/2022
DOI: 10.1016/j.jaip.2022.01.042
PMID: 35172220
Abstract
Optimal management of patients with combined immunodeficiency, especially pertaining to hematopoietic stem cell transplantation (HSCT), remains unclear.
To identify factors influencing HSCT and mortality in the population with combined immunodeficiency in North America.
We identified 337 participants in the United States Immunodeficiency Network database with diverse forms of combined immunodeficiency and their characteristics, including demographic characteristics, laboratory values, infectious history, comorbidities, and treatment strategies. Univariate analysis was performed using logistic regression, whereas multivariate analysis was performed using multiple Cox proportional hazards.
On univariate analysis, disseminated invasive viral infections and variants in STAT3, GATA2, and, DOCK8 were associated with increased odds of HSCT. Mucocutaneous fungal infections and variants in STAT3 were associated with increased odds of survival, whereas disseminated/invasive fungal infections, disseminated/invasive viral infections, and parasitic infections were associated with decreased odds of survival. On multiple variable Cox proportional hazards analysis, variants in ZAP70, nonspecific bacterial, and disseminated/invasive viral infections were associated with increased hazards of transplantation, whereas variants in multiple genes (RMRP, NEMO, DOCK8, CD40L, and CARD9), disseminated/invasive viral infections, autoimmune disease, and higher absolute lymphocyte count were associated with increased hazards of death. Importantly, demographic characteristics, basic lymphocyte subset counts, and absence of genetic diagnosis were not associated with HSCT or mortality.
We determined that specific genetic diagnoses and infection burden impacts the decision to undergo HSCT in this cohort. In addition, certain genetic diagnoses and invasive viral infections carry an increased risk of mortality.
Details
- Title: Subtitle
- Morbidity, Mortality, and Therapeutics in Combined Immunodeficiency: Data From the USIDNET Registry
- Creators
- Jessica Durkee-Shock - National Institute of Allergy and Infectious DiseasesAnqing Zhang - George Washington UniversityHua Liang - George Washington UniversityHannah Wright - United States Immunodeficiency NetworkJulieann Magnusson - United States Immunodeficiency NetworkElizabeth Garabedian - National Human Genome Research InstituteRebecca A. Marsh - Cincinnati Children's Hospital Medical CenterKathleen E. Sullivan - Children's Hospital of PhiladelphiaMichael D. Keller - George Washington UniversityJennifer PuckElizabeth SecordJaveed AkhterTamara PozosRamsay FuleihanKarin ChenRebecca BuckleyNiraj PatelDaniel SuezMegan CooperManish ButteFrancisco BonillaKelly WalkovichElie HaddadCharlotte Cunningham-RundlesGary KleinerHey ChongZuhair BallasBurcin UygungilVivian Hernandez-TrujilloElizabeth A. SecordNicholas HartogMorna DorseyRalph ShapiroSusan SchuvalLuigi NotarangeloJohn RoutesAdina KnightNicholas BennettFatima KhanJolan WalterChristine SeroogyHans OchsKathleen HainesMica MuskatPatricia Costa ReisLaurence ChengUSIDNET Consortium
- Resource Type
- Journal article
- Publication Details
- The journal of allergy and clinical immunology in practice (Cambridge, MA), Vol.10(5), pp.1334-1341.e6
- Publisher
- Elsevier Inc
- DOI
- 10.1016/j.jaip.2022.01.042
- PMID
- 35172220
- ISSN
- 2213-2198
- eISSN
- 2213-2201
- Grant note
- The United States Immunodeficiency Network U24AI86837 / Immune Deficiency Foundation Jeffrey Modell Foundation NIAID
- Language
- English
- Date published
- 05/2022
- Academic Unit
- Immunology; Internal Medicine
- Record Identifier
- 9984360047502771
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