Tshimanga S1*, Fouad A1, Fakiri K1, Rada N1, Draiss G1, Edmou B2, Bouskraoui M1
1Pediatrics Department, Mohammed VI University Hospital, Marrakesh, Morocco
2Immunology Department, Mohammed VI University Hospital, Marrakesh, Morocco
*Corresponding Author: Stanislas Tshimanga, Pediatrics Department, Mohammed VI University Hospital, PB 2360, Ibn Sina Avenue, Marrakesh, Morocco, Tel/Fax: +212524300700; E-mail: [email protected].
Received Date: February 03, 2021
Published Date: March 13, 2021
Citation: Tshimanga S, Fouad A, Fakiri K, Rada N, Draiss G, Edmou B. (2021). Reticular Dysgenesis: Fiction or Reality in Morocco. Mathews J Immunol Allergy. (5)1:12.
Copyrights: Tshimanga S, et al. © 2021. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
ABSTRACT
Severe combined immune deficits (SCID) are a group of rare yet the severest forms of primitive immune deficits. Reticular dysgenesis (RD) is a rare form of SCID characterized by lack of circulating T lymphocytes, severe congenital neutropenia, and sensorineural deafness. Mutations of the gene coding for adenylate kinase2 (AK2) have been identified as the molecular basis, and treatment consists in bone marrow transplantation. In this article, we report a case of reticular dysgenesis, a very rare condition in Morocco, and a review of the literature.