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Defining the Molecular Requirements for Human Lymphopoiesis

Hematopoietic stem cells (HSCs) are defined by their self‐renewal capacity and their ability to contribute to the pool of differentiated cells that ensure immune regulation and protective immunity. In a recent STEM CELLS review, researchers led by Stuart G. Tangye (University of New South Wales, Australia) discuss how the comprehensive study of patients harboring rare germline genetic defects that result in immune dysfunctions and an increased susceptibility to infectious disease (collectively named primary immunodeficiencies) have enabled a better understanding of the intricate process of lymphocyte development in human, improved diagnosis, and facilitated the development of curative rather than supportive treatments for patients with primary immunodeficiencies.