Aberrant T-cell exhaustion in severe combined immunodeficiency survivors with poor T-cell reconstitution after transplantation.

ABSTRACT:

Background

Severe combined immunodeficiency (SCID) comprises rare inherited disorders of immunity that require definitive treatment through hematopoietic cell transplantation (HCT) or gene therapy for survival. Despite successes of allogeneic HCT, many SCID patients experience incomplete immune reconstitution, persistent T-cell lymphopenia, and poor long-term outcomes.

Objective

We hypothesized that CD4⁺ T-cell lymphopenia could be associated with a state of T-cell exhaustion in previously transplanted SCID patients.

Methods

We analyzed markers of exhaustion in blood samples from 61 SCID patients at a median of 10.4 years after HCT.

Results

Compared to post-HCT SCID patients with normal CD4⁺ T-cell counts, those with poor T-cell reconstitu

SUBMITTER: Labrosse R 

PROVIDER: S-EPMC9924130 | biostudies-literature | 2023 Jan

REPOSITORIES: biostudies-literature