CD40 ligand deficiency causes functional defects of peripheral neutrophils that are improved by exogenous IFN-γ.

ABSTRACT:

Background

Patients with X-linked hyper-IgM syndrome caused by CD40 ligand (CD40L) deficiency often present with episodic, cyclic, or chronic neutropenia, suggesting abnormal neutrophil development in the absence of CD40L-CD40 interaction. However, even when not neutropenic and despite immunoglobulin replacement therapy, CD40L-deficient patients are susceptible to life-threatening infections caused by opportunistic pathogens, suggesting impaired phagocyte function and the need for novel therapeutic approaches.

Objectives

We sought to analyze whether peripheral neutrophils from CD40L-deficient patients display functional defects and to explore the in vitro effects of recombinant human IFN-γ (rhIFN-γ) on neutrophil function.

Methods

We investigated the microbicidal acti

SUBMITTER: Cabral-Marques O 

PROVIDER: S-EPMC6123297 | biostudies-literature | 2018 Nov

REPOSITORIES: biostudies-literature