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Clinical and Immunological Features of 96 Moroccan Children with SCID Phenotype: Two Decades’ Experience

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Abstract

Severe combined immunodeficiency (SCID) is a heterogeneous group of primary immunodeficiency diseases (PIDs) characterized by a lack of autologous T lymphocytes. This severe PID is rare, but has a higher prevalence in populations with high rates of consanguinity. The epidemiological, clinical, and immunological features of SCIDs in Moroccan patients have never been reported. The aim of this study was to provide a clinical and immunological description of SCID in Morocco and to assess changes in the care of SCID patients over time. This cross-sectional retrospective study included 96 Moroccan patients referred to the national PID reference center at Casablanca Children’s Hospital for SCID over two decades, from 1998 to 2019. The case definition for this study was age < 2 years, with a clinical phenotype suggestive of SCID, and lymphopenia, with very low numbers of autologous T cells, according to the IUIS Inborn Errors of Immunity classification. Our sample included 50 male patients, and 66% of the patients were born to consanguineous parents. The median age at onset and diagnosis were 3.3 and 6.5 months, respectively. The clinical manifestations commonly observed in these patients were recurrent respiratory tract infection (82%), chronic diarrhea (69%), oral candidiasis (61%), and failure to thrive (65%). The distribution of SCID phenotypes was as follows: T−B−NK+ in 44.5%, T−B−NK− in 32%, T−B+NK− in 18.5%, and T−B+NK+ in 5%. An Omenn syndrome phenotype was observed in 15 patients. SCID was fatal in 84% in the patients in our cohort, due to the difficulties involved in obtaining urgent access to hematopoietic stem cell transplantation, which, nevertheless, saved 16% of the patients. The autosomal recessive forms of the clinical and immunological phenotypes of SCID, including the T−B−NK+ phenotype in particular, were more frequent than those in Western countries. A marked improvement in the early detection of SCID cases over the last decade was noted. Despite recent progress in SCID diagnosis, additional efforts are required, for genetic confirmation and particularly for HSCT.

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Data Availability

The datasets generated during and/or analyzed during the current study are available from the corresponding author on reasonable request.

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Acknowledgments

We would like to thank Dr. Ibrahim Khalil Ahmadaye for the excellent help with statistics. We would like to thank the Hajar Association, which provides research funding and a network for PID patients. We would also like to thank Prof. Allessandro Aiuiti and his team for providing genetic results.

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Authors and Affiliations

  1. Clinical Immunology Unit, Infectious Diseases Department, Children’s Hospital, Ibn Rochd University Hospital, Casablanca, Morocco

    Ibtihal Benhsaien, Fatima Ailal, Naïma Amenzoui, Zineb Jouhadi & Ahmed Aziz Bousfiha

  2. Cellular and Molecular Pathology Laboratory, Faculty of Medicine and Pharmacy, Hassan II University, Casablanca, Morocco

    Ibtihal Benhsaien & Abdallah Badou

  3. Clinical Immunology, Autoimmunity and Inflammation Laboratory (LICIA), Faculty of Medicine and Pharmacy, Hassan II University, Casablanca, Morocco

    Ibtihal Benhsaien, Fatima Ailal, Jalila El Bakkouri, Leïla Jeddane, Hind Ouair & Ahmed Aziz Bousfiha

  4. Immunology Laboratory, Ibn Rochd University Hospital, Casablanca, Morocco

    Jalila El Bakkouri

  5. Laboratoire National de Référence, Mohamed VI Health Sciences University, Casablanca, Morocco

    Leïla Jeddane

  6. Immunology Laboratory, Mohammed VI University Hospital, Marrakech, Morocco

    Brahim Admou

  7. Department of Pediatric Infectious Diseases, Mohammed VI University Hospital, Marrakech, Morocco

    Mohamed Bouskraoui & Nouredine Rada

  8. Department of Pediatric Diseases, Hassan II University Hospital, Fes, Morocco

    Mohamed Hbibi & Mustapha Hida

  9. Department of Pediatric Infectious Diseases, Avicenna University Hospital, Rabat, Morocco

    Naïma El Hafidi

  10. Department of Pediatrics, Mohammed VI University Hospital, Oujda, Morocco

    Noufissa Benajiba

  11. Department of Pediatrics, Mohammed V Military Hospital, Faculty of Medicine and Pharmacy, Mohammed V University, Rabat, Morocco

    Rachid Abilkassem

Authors
  1. Ibtihal Benhsaien
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  2. Fatima Ailal
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  3. Jalila El Bakkouri
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  4. Leïla Jeddane
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  5. Hind Ouair
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  6. Brahim Admou
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  7. Mohamed Bouskraoui
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  8. Mohamed Hbibi
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  9. Mustapha Hida
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  10. Naïma Amenzoui
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  11. Zineb Jouhadi
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  12. Naïma El Hafidi
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  13. Nouredine Rada
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  14. Noufissa Benajiba
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  15. Rachid Abilkassem
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  16. Abdallah Badou
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  17. Ahmed Aziz Bousfiha
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Contributions

I. Benhsaien: Performed the analysis, interpreted data, and wrote the manuscript.

F. Ailal: Managed patients, interpreted data, and reviewed the manuscript.

J. El Bakkouri: Performed immunological analysis and provided material from patients and reagents.

L. Jeddane: Performed immunological analysis.

H. Ouair: Provided material from patients and reagents.

B. Admou: Performed analysis.

M. Bouskraoui: Provided data from patients.

MM. Hbibi: Provided data from patients.

M. Hida: Provided data from patients.

N. Amenzoui: Provided data from patients.

Z. Jouhadi: Provided data from patients.

N. Rada: Managed patients and interpreted data.

N. Benajiba: Managed patients and interpreted data.

R. Abilkacim: Managed patients and interpreted data.

N. Elhafidi: Managed patients and interpreted data.

A. Badou: Designed the study, interpreted data, and edited the manuscript.

AA. Bousfiha: Conceived the study, and edited the manuscript; corresponding author.

Corresponding author

Correspondence to Ahmed Aziz Bousfiha.

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Benhsaien, I., Ailal, F., El Bakkouri, J. et al. Clinical and Immunological Features of 96 Moroccan Children with SCID Phenotype: Two Decades’ Experience. J Clin Immunol 41, 631–638 (2021). https://doi.org/10.1007/s10875-020-00960-x

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  • DOI: https://doi.org/10.1007/s10875-020-00960-x

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