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Abstract
RÉSUMÉ
Introduction. La thrombopénie pédiatrique est fréquemment attribuée à des causes infectieuses communes comme le paludisme. Cependant, elle peut constituer un signe précoce de déficit immunitaire primitif (DIP), une entité sous-diagnostiquée en Afrique subsaharienne. Une identification rapide des DIP est cruciale pour améliorer le pronostic. L’objectif de cette étude était d’analyser le profil des thrombopénies chez l’enfant hospitalisé à Abidjan et d’identifier les signes clinico-biologiques associés à une suspicion de DIP. Méthodologie. Une étude analytique a été menée en 2024 au service de pédiatrie du CHU de Cocody. Tous les enfants âgés de 1 mois à 15 ans hospitalisés avec une thrombopénie documentée ont été inclus. Les données démographiques, cliniques, biologiques et évolutives ont été collectées. Les facteurs associés à une suspicion de DIP ont été analysés par des tests statistiques appropriés. Résultats. Parmi 809 admissions, 104 enfants présentaient une thrombopénie (prévalence hospitalière de 12,9%). L’âge moyen était de 55,7 mois et le sex-ratio de 2,05. Un DIP a été suspecté chez 21 patients (20,2%). Le paludisme (73,1%) était le diagnostic principal suivi des infections bactériennes (25%). La suspicion de DIP n’était pas liée à l’âge, au sexe ou à la consanguinité (prévalance : 20.2%). En revanche, elle était fortement associée à certaines anomalies hématologiques : la présence d’une leucopénie (OR=16,4 ; p<0,0001) ou d’une neutropénie (OR=16,4 ; p<0,0001) multipliait par plus de 16 le risque de suspicion. La pancytopénie était le facteur de risque le plus puissant, l’augmentant par un facteur de 41 (OR=41,0 ; p<0,0001). Conclusion. Au-delà des causes infectieuses dominantes, cette étude identifie des signes d’alerte hématologiques majeurs pour les DIP chez l’enfant thrombopénique. La découverte d’une neutropénie, d’une leucopénie, et surtout d’une pancytopénie, doit systématiquement déclencher un bilan immunologique approfondi pour permettre un diagnostic et une prise en charge spécialisée précoces.
ABSTRACT
Introduction. Pediatric thrombocytopenia is often attributed to common infectious causes like malaria. However, it can be an early sign of primary immunodeficiency (PID), an underdiagnosed entity in sub-Saharan Africa. Rapid identification of PID is crucial to improve prognosis. This study aimed to analyze the profile of thrombocytopenia in hospitalized children in Abidjan and identify the clinico-biological signs associated with a suspicion of PID. Methodology. An analytical study was conducted in 2024 in the pediatric department of Cocody University Hospital. All children aged 1 month to 15 years hospitalized with documented thrombocytopenia were included. Demographic, clinical, biological, and outcome data were collected. Factors associated with a suspicion of PID were analyzed using appropriate statistical tests. Results. Among 809 admissions, 104 children had thrombocytopenia (hospital prevalence of 12.9%). The mean age was 55.7 months and the sex ratio was 2.05. PID was suspected in 21 patients (20.2%). Malaria was the main diagnosis (73.1%), followed by bacterial infections (25%). Suspicion of PID was not linked to age, sex, or consanguinity (prevalence: 20.2%). In contrast, it was strongly associated with specific hematological abnormalities: the presence of leukopenia (OR=16.4; p<0.0001) or neutropenia (OR=16.4; p<0.0001) increased the risk of suspicion more than 16-fold. Pancytopenia was the strongest risk factor, increasing it by a factor of 41 (OR=41.0; p<0.0001). Conclusion. Beyond the dominant infectious causes, this study identifies major hematological warning signs for PID in thrombocytopenic children. The finding of neutropenia, leukopenia, and especially pancytopenia, should systematically trigger an in-depth immunological workup to enable early diagnosis and specialized management.
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References
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- 4. Seidel MG, Allenspach EJ, Grosse S. Autoimmune cytopenias in PID: Pathophysiology and treatment. Blood. 2019;133(6):551-559.
- 5. Bousfiha A, Jeddane L, Ailal F, et al. Primary immunodeficiency diseases in Africa. Curr Opin Allergy Clin Immunol. 2017;17(6):590-601.
- 6. Rezaei N, Fadaei R, et al. Approach to the management of autoimmunity in primary immunodeficiency. J Allergy Clin Immunol. 2011;127(2):378-385.
- 7. Pai SY, Allmond L, Jolles S. Transplantation outcomes for primary immunodeficiencies. J Allergy Clin Immunol. 2018;141(3):871-879.
- 8. Neunert C, Lim W, Crowther M, Cohen A, Solberg LA, Neufeld EJ, et al. Management of newly diagnosed immune thrombocytopenia in children and adults: An international consensus report. Blood Adv. 2019;3(4):1057-1067.
- 9. Ostrowski S, Liebhaber M, Vengrol J, et al. Prevalence and causes of thrombocytopenia in a pediatric population. J Pediatr. 2012;161(3):441-447.
- 10. Brousse M, Martin J, Ferron F, et al. Sex differences in pediatric thrombocytopenia. Pediatr Blood Cancer. 2014;61(7):1324-1329.
- 11. Giansily-Blaizot M, Pham A, Saad N, et al. Pancytopenia in children: Causes and prognosis. Eur J Pediatr Hematol. 2013;45(5):347-352.
- 12. Bousfiha A, Jeddane L, Ailal F, et al. The ever-expanding clinical spectrum of primary immunodeficiencies. Int Rev Immunol. 2020;39(1):1-14.
- 13. Picard C, Al-Herz W, Bousfiha A, et al. Primary immunodeficiencies: 2018 update. J Clin Immunol. 2018;38(1):1-24.
- 14. Tangye SG, Al-Herz W, Bousfiha A, et al. Human inborn errors of immunity: 2019 update on the classification from the International Union of Immunological Societies. J Clin Immunol. 2020;40(1):24-61.
- 15. Fioredda F, Fagioli F, Messina C, et al. Neutropenia as a marker of primary immunodeficiency in children: A retrospective cohort study. Pediatr Blood Cancer. 2019;66(2):e27668.
References
1. Rodeghiero F, Stasi R, Gernsheimer T, Michel M, Cines DB, McMillan R. Standardization of terminology, definitions, and outcome criteria in immune thrombocytopenic purpura. Blood. 2009;113(11):2386-2393.
2. Tangye SG, Al-Herz W, Bousfiha A, Cunningham-Rundles C, Geha RS, Gennery AR, et al. Human inborn errors of immunity: 2022 IUIS update. J Clin Immunol. 2022;42(7):1473-1507.
3. Picard C, Al-Herz W, Bousfiha A, Casanova JL, Chapgier A, Cunningham-Rundles C, et al. Primary immunodeficiencies associated with autoimmunity: From bench to bedside. Curr Opin Immunol. 2016;43:25-34.
4. Seidel MG, Allenspach EJ, Grosse S. Autoimmune cytopenias in PID: Pathophysiology and treatment. Blood. 2019;133(6):551-559.
5. Bousfiha A, Jeddane L, Ailal F, et al. Primary immunodeficiency diseases in Africa. Curr Opin Allergy Clin Immunol. 2017;17(6):590-601.
6. Rezaei N, Fadaei R, et al. Approach to the management of autoimmunity in primary immunodeficiency. J Allergy Clin Immunol. 2011;127(2):378-385.
7. Pai SY, Allmond L, Jolles S. Transplantation outcomes for primary immunodeficiencies. J Allergy Clin Immunol. 2018;141(3):871-879.
8. Neunert C, Lim W, Crowther M, Cohen A, Solberg LA, Neufeld EJ, et al. Management of newly diagnosed immune thrombocytopenia in children and adults: An international consensus report. Blood Adv. 2019;3(4):1057-1067.
9. Ostrowski S, Liebhaber M, Vengrol J, et al. Prevalence and causes of thrombocytopenia in a pediatric population. J Pediatr. 2012;161(3):441-447.
10. Brousse M, Martin J, Ferron F, et al. Sex differences in pediatric thrombocytopenia. Pediatr Blood Cancer. 2014;61(7):1324-1329.
11. Giansily-Blaizot M, Pham A, Saad N, et al. Pancytopenia in children: Causes and prognosis. Eur J Pediatr Hematol. 2013;45(5):347-352.
12. Bousfiha A, Jeddane L, Ailal F, et al. The ever-expanding clinical spectrum of primary immunodeficiencies. Int Rev Immunol. 2020;39(1):1-14.
13. Picard C, Al-Herz W, Bousfiha A, et al. Primary immunodeficiencies: 2018 update. J Clin Immunol. 2018;38(1):1-24.
14. Tangye SG, Al-Herz W, Bousfiha A, et al. Human inborn errors of immunity: 2019 update on the classification from the International Union of Immunological Societies. J Clin Immunol. 2020;40(1):24-61.
15. Fioredda F, Fagioli F, Messina C, et al. Neutropenia as a marker of primary immunodeficiency in children: A retrospective cohort study. Pediatr Blood Cancer. 2019;66(2):e27668.
