Predictors of early death risk among untransplanted patients with combined immunodeficiencies affecting cellular and humoral immunity: A multicenter report

Waleed Al-Herz, Ali H. Ziyab, Mehdi Adeli, Tariq Al Farsi, Suleiman Al-Hammadi, Amna Ali Al Kuwaiti, Maryam Al-Nesf, Nashat Al Sukaiti, Salem Al-Tamemi, Hiba Shendi

Research output: Contribution to journalArticlepeer-review

Abstract

Background: There is an increased demand for hematopoietic stem cell transplant (HSCT) to treat various diseases including combined immunodeficiencies (CID), with limited worldwide availability. Variables affecting the decision regarding CID patients' prioritization for HSCT are not known. We aimed to determine general, clinical, and immunologic factors associated with the higher risk of early death (≤6 months after diagnosis) in untransplanted CID patients. Methods: Data collection was done retrospectively from five centers and included general patients' information, and clinical and laboratory variables. Inclusion criteria were untransplanted patients who are either dead or alive with a follow-up period ≥6 months after diagnosis. Results: Two hundred and thirty-six CID patients were reported by participating centers, of whom 111 were included in the study with a cumulative follow-up period of 278.6 years. Seventy-two patients died with the median age of death of 10.5 months. 35.1% of the patients succumbed within 6 months after the diagnosis. Having a history of Candida infections, sepsis or hepatomegaly was associated with an increased risk of early death. None of the other general or clinical variables was associated with such risk. Bivariate analysis of lymphocyte subsets showed that patients with the following counts: CD3+ < 100, CD4+ < 200, CD8+ < 50, or CD16+CD56+ <200 cells/μl had increased risk of early death. In adjusted analysis, increased risk of early death was observed among patients with CD3+ count <100 cells/μl. Conclusion: Combined immunodeficiencies patients with a history of Candida infections, sepsis, hepatomegaly, or severe T-lymphopenia should be given priority for HSCT to avoid early death.

Original languageEnglish
Article numbere13901
JournalPediatric Allergy and Immunology
Volume33
Issue number12
DOIs
Publication statusPublished - Dec 2022

Keywords

  • combined immunodeficiency
  • death
  • epidemiology
  • hematopoietic stem cell transplant
  • inborn error of immunity
  • infections
  • newborn screening
  • outcome
  • survival

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Immunology and Allergy
  • Immunology

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