A Registry Study of 240 Patients with X-Linked Agammaglobulinemia Living in the USA.

ABSTRACT:

Purpose

To understand the natural history and clinical outcomes for patients with X-linked agammaglobulinemia (XLA) in the United States utilizing the United States Immunodeficiency Network (USIDNET) patient registry.

Methods

The USIDNET registry was queried for data from XLA patients collected from 1981 to 2019. Data fields included demographics, clinical features before and after diagnosis of XLA, family history, genetic mutation in Bruton's tyrosine kinase (BTK), laboratory findings, treatment modalities, and mortality.

Results

Data compiled through the USIDNET registry on 240 patients were analyzed. Patient year of birth ranged from 1945 to 2017. Living status was available for 178 patients; 158/178 (88.8%) were alive. Race was reported for 204 patients as follows

SUBMITTER: Hernandez-Trujillo V 

PROVIDER: S-EPMC10354121 | biostudies-literature | 2023 Aug

REPOSITORIES: biostudies-literature