Epidemiology of combined immunodeficiencies affecting cellular and humoral immunity– a multicentric retrospective cohort study from the Arabian Peninsula

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

doi:10.1016/j.clim.2023.109696

Epidemiology of combined immunodeficiencies affecting cellular and humoral immunity– a multicentric retrospective cohort study from the Arabian Peninsula

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 journal › Article › peer-review

Abstract

Aims: To understand the characteristics of combined immunodeficiency disorders that affect cellular and humoral immunity (CID) in the Arabian Peninsula. Methods: Retrospective study of 236 patients with CID from the region were enrolled from 2004 to 2022. Results: 236 patients were included with a majority being profound CID. Among patients with a family history of CID, the ages at onset and diagnosis, and the delay in diagnosis were lower compared to those with no family history of CID, but this did not affect time to transplant. HSCT was performed for 51.27% of the patients with median time from diagnosis to HSCT of 6.36 months. On multivariate analysis, patients who underwent early transplant had increased odds of having CD3 count ≤1000 cell/μl, diagnosed by screening or erythroderma. Conclusion: There is a delay in diagnosis and treatment of CID in our region. Establishing newborn screening programs and HSCT units in our region are the urgent need.

Original language	English
Article number	109696
Journal	Clinical Immunology
Volume	254
DOIs	https://doi.org/10.1016/j.clim.2023.109696
Publication status	Published - Sept 2023
Externally published	Yes

Keywords

Combined immunodeficiency disorders (CID)
Epidemiology
Genetics
Hematopoietic stem cell transplant
Mortality
Newborn screening

ASJC Scopus subject areas

Immunology and Allergy
Immunology

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10.1016/j.clim.2023.109696

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Al-Herz, W., Ziyab, A. H., Adeli, M., Al Farsi, T., Al-Hammadi, S., Al Kuwaiti, A. A., Al-Nesf, M., Al Sukaiti, N., Al-Tamemi, S., & Shendi, H. (2023). Epidemiology of combined immunodeficiencies affecting cellular and humoral immunity– a multicentric retrospective cohort study from the Arabian Peninsula. Clinical Immunology, 254, Article 109696. https://doi.org/10.1016/j.clim.2023.109696

Al-Herz, W, Ziyab, AH, Adeli, M, Al Farsi, T, Al-Hammadi, S, Al Kuwaiti, AA, Al-Nesf, M, Al Sukaiti, N, Al-Tamemi, S & Shendi, H 2023, 'Epidemiology of combined immunodeficiencies affecting cellular and humoral immunity– a multicentric retrospective cohort study from the Arabian Peninsula', Clinical Immunology, vol. 254, 109696. https://doi.org/10.1016/j.clim.2023.109696

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abstract = "Aims: To understand the characteristics of combined immunodeficiency disorders that affect cellular and humoral immunity (CID) in the Arabian Peninsula. Methods: Retrospective study of 236 patients with CID from the region were enrolled from 2004 to 2022. Results: 236 patients were included with a majority being profound CID. Among patients with a family history of CID, the ages at onset and diagnosis, and the delay in diagnosis were lower compared to those with no family history of CID, but this did not affect time to transplant. HSCT was performed for 51.27% of the patients with median time from diagnosis to HSCT of 6.36 months. On multivariate analysis, patients who underwent early transplant had increased odds of having CD3 count ≤1000 cell/μl, diagnosed by screening or erythroderma. Conclusion: There is a delay in diagnosis and treatment of CID in our region. Establishing newborn screening programs and HSCT units in our region are the urgent need.",

keywords = "Combined immunodeficiency disorders (CID), Epidemiology, Genetics, Hematopoietic stem cell transplant, Mortality, Newborn screening",

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

note = "Funding Information: This work was support by a publication grant from CSL Behring with no role in the development of the manuscript or the decision to publish. Publisher Copyright: {\textcopyright} 2023 Elsevier Inc.",

year = "2023",

month = sep,

doi = "10.1016/j.clim.2023.109696",

language = "English",

volume = "254",

journal = "Clinical Immunology",

issn = "1521-6616",

publisher = "Academic Press Inc.",

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T1 - Epidemiology of combined immunodeficiencies affecting cellular and humoral immunity– a multicentric retrospective cohort study from the Arabian Peninsula

AU - Al-Herz, Waleed

AU - Ziyab, Ali H.

AU - Adeli, Mehdi

AU - Al Farsi, Tariq

AU - Al-Hammadi, Suleiman

AU - Al Kuwaiti, Amna Ali

AU - Al-Nesf, Maryam

AU - Al Sukaiti, Nashat

AU - Al-Tamemi, Salem

AU - Shendi, Hiba

N1 - Funding Information: This work was support by a publication grant from CSL Behring with no role in the development of the manuscript or the decision to publish. Publisher Copyright: © 2023 Elsevier Inc.

PY - 2023/9

Y1 - 2023/9

N2 - Aims: To understand the characteristics of combined immunodeficiency disorders that affect cellular and humoral immunity (CID) in the Arabian Peninsula. Methods: Retrospective study of 236 patients with CID from the region were enrolled from 2004 to 2022. Results: 236 patients were included with a majority being profound CID. Among patients with a family history of CID, the ages at onset and diagnosis, and the delay in diagnosis were lower compared to those with no family history of CID, but this did not affect time to transplant. HSCT was performed for 51.27% of the patients with median time from diagnosis to HSCT of 6.36 months. On multivariate analysis, patients who underwent early transplant had increased odds of having CD3 count ≤1000 cell/μl, diagnosed by screening or erythroderma. Conclusion: There is a delay in diagnosis and treatment of CID in our region. Establishing newborn screening programs and HSCT units in our region are the urgent need.

AB - Aims: To understand the characteristics of combined immunodeficiency disorders that affect cellular and humoral immunity (CID) in the Arabian Peninsula. Methods: Retrospective study of 236 patients with CID from the region were enrolled from 2004 to 2022. Results: 236 patients were included with a majority being profound CID. Among patients with a family history of CID, the ages at onset and diagnosis, and the delay in diagnosis were lower compared to those with no family history of CID, but this did not affect time to transplant. HSCT was performed for 51.27% of the patients with median time from diagnosis to HSCT of 6.36 months. On multivariate analysis, patients who underwent early transplant had increased odds of having CD3 count ≤1000 cell/μl, diagnosed by screening or erythroderma. Conclusion: There is a delay in diagnosis and treatment of CID in our region. Establishing newborn screening programs and HSCT units in our region are the urgent need.

KW - Combined immunodeficiency disorders (CID)

KW - Epidemiology

KW - Genetics

KW - Hematopoietic stem cell transplant

KW - Mortality

KW - Newborn screening

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Epidemiology of combined immunodeficiencies affecting cellular and humoral immunity– a multicentric retrospective cohort study from the Arabian Peninsula

Abstract

Keywords

ASJC Scopus subject areas

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