Family History of Agammaglobulinemia Among Racial and Ethnic Minority Groups with X-linked Agammaglobulinemia from the USIDNET Registry

Abstract

BackgroundStudies have demonstrated health disparities among racial and ethnic minorities but there is limited data regarding patients with primary immunodeficiencies. A positive family history of agammaglobulinemia has been hypothesized as a potential factor to support improved outcomes in patients with XLA secondary to increased family and clinician awareness. ObjectiveWe report on family history of agammaglobulinemia among racial and ethnic groups with X-linked agammaglobulinemia (XLA) from the U.S. Immunodeficiency Network (USIDNET) Registry. MethodsPatients with agammaglobulinemia and an investigator designated BTK defect were included. Family history of agammaglobulinemia was reported among racial and ethnic groups with XLA. Positive family history of agammaglobulinemia was compared between racial and ethnic groups using a Fisher’s exact tests. ResultsOf 231 XLA patients included, 140 (60.6%) identified as White, 20 (8.7%) identified as Black or African American, 15 (6.5%) identified as Hispanic or Latino, 6 (2.6%) identified as Asian or Pacific Islander, and 50 (21.6%) identified as other, more than one, or unknown for race and/or ethnicity. Of 93 patients with an identified race and/or ethnicity reported to have a positive family history of agammaglobulinemia, 69 (74.2%) identified as White, 12 (12.9%) as Black or African American, 10 (10.7%) as Hispanic or Latino, and 2 (2.2%) as Asian or Other Pacific Islander. Of 52 patients with an identified race or ethnicity reported to have a negative family history of agammaglobulinemia, 41 (78.8%) identified as White, 4 (7.7%) as Black or African American, 3 (5.8%) as Hispanic or Latino, and 4 (7.7%) as Asian or Pacific Islander. Of the 181 patients with an identified race and/or ethnicity, 36 (19.9%) were reported as unknown or not reported for family history of agammaglobulinemia. Compared 69 (49.3%) White patients, Non-White patients including 12 (60.0%) Black or African American, 10 (66.7%) Hispanic or Latino, and 2 (33.3%) Asian or Pacific Islander patients with XLA were reported to have a positive family history of agammaglobulinemia (p = 0.69). ConclusionsWithin the USIDNET Registry database, we did not identify a statistically significant difference in positive family history of agammaglobulinemia among racial and ethnic groups in patients with XLA.