Exaggerated follicular helper T-cell responses in patients with LRBA deficiency caused by failure of CTLA4-mediated regulation.

ABSTRACT:

Background

LPS-responsive beige-like anchor protein (LRBA) and cytotoxic T lymphocyte-associated antigen 4 (CTLA4) deficiencies give rise to overlapping phenotypes of immune dysregulation and autoimmunity, with dramatically increased frequencies of circulating follicular helper T (cT_FH) cells.

Objective

We sought to determine the mechanisms of cT_FH cell dysregulation in patients with LRBA deficiency and the utility of monitoring cT_FH cells as a correlate of clinical response to CTLA4-Ig therapy.

Methods

cT_FH cells and other lymphocyte subpopulations were characterized. Functional analyses included in vitro follicular helper T (T_FH) cell differentiation and cT_FH/naive B-cell cocultures. Serum soluble IL-

SUBMITTER: Alroqi FJ 

PROVIDER: S-EPMC5743769 | biostudies-literature | 2018 Mar

REPOSITORIES: biostudies-literature