Profil immunophénotypique des déficits immunitaires combinés sévères au Maroc

Abstract

Summary Objective Severe combined immunodeficiency (SCID) is a group of disorders that leads to early childhood death as a result of severe infections. High consanguineous rate in Maghreb, from 19.9 % in Morocco to 22.6 % in Algeria, allow us to suggest a high prevalence of autosomal recessive SCIDs. Methods We analysed a series of 30 SCID, recruited on a 12 years period (1997–2008). Analysis included a blood count, Immunoglobulins A, G and M dosage by Mancini's radial immunodiffusion and lymphocyte subpopulations count by flow cytometry. Results On the 30 SCID, 19 were T−B− SCID and 11 T−B+ SCID. NK counts were available for only 25 patients. Fourteen patients were T−B−NK+, two were T−B−NK−, six T−B−NK+ and three T−B+NK−. Conclusion Our results show a predominance of T−B−NK+ SCID in our series, probably due to RAG1/2 deficiency. A molecular analysis is yet to be done to confirm this hypothesis.