Reticular Dysgenesis: Fiction or Reality in Morocco

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.