Nodular regenerative hyperplasia in X-linked agammaglobulinemia: An underestimated and severe complication.

ABSTRACT:

Background

Late-onset complications in X-linked agammaglobulinemia (XLA) are increasingly recognized. Nodular regenerative hyperplasia (NRH) has been reported in primary immunodeficiency but data in XLA are limited.

Objectives

This study sought to describe NRH prevalence, associated features, and impact in patients with XLA.

Methods

Medical records of all patients with XLA referred to the National Institutes of Health between October 1994 and June 2019 were reviewed. Liver biopsies were performed when clinically indicated. Patients were stratified into NRH+ or NRH- groups, according to their NRH biopsy status. Fisher exact test and Mann-Whitney test were used for statistical comparisons.

Results

Records of 21 patients with XLA were reviewed, with a cumulative f

SUBMITTER: Nunes-Santos CJ 

PROVIDER: S-EPMC8633079 | biostudies-literature | 2022 Jan

REPOSITORIES: biostudies-literature