Humanized mouse models reveal an immunologic classification of idiopathic CD4 lymphocytopenia subtypes.

ABSTRACT: Idiopathic CD4 lymphocytopenia (ICL) is a clinically heterogeneous immunodeficiency disorder defined by low numbers of circulating CD4+ T cells and increased susceptibility to opportunistic infections. CD8+ T cells, NK, and/or B cells may also be deficient in some patients. To delineate possible pathogenic cellular mechanisms in ICL, we compared immune system development and function in NOD-RAGKO-γcKO (NRG) mice transplanted with hematopoietic stem cells from patients with ICL or healthy controls. CD34+ hematopoietic stem cells from healthy controls and patients with ICL reconstituted NRG mice equally well. In contrast, PBMC transfers into NRG mice identified 2 ICL engraftment phenotypes, reconstituting and nonreconstituting (NR), based on the absence or presence of donor lymphopenia. For

SUBMITTER: Perez-Diez A 

PROVIDER: S-EPMC6675561 | biostudies-literature | 2019 Jul

REPOSITORIES: biostudies-literature