The Evaluation of Neutropenia in X-linked Agammaglobulinemia  Patients

Molood   Safarirad; Ali  Abbaszadeh Ganji; Ahmad  Vosughi Motlagh

doi:10.18502/igj.v3i4.7459

Molood Safarirad Department of Pediatrics, North Khorasan University of Medical Sciences, Bojnurd, Iran.
Ali Abbaszadeh Ganji Student Research Committee of North Khorasan University of Medical Sciences, Bojnurd, Iran.
Ahmad Vosughi Motlagh Department of Pediatrics, North Khorasan University of Medical Sciences, Bojnurd, Iran.

DOI: https://doi.org/10.18502/igj.v3i4.7459

Keywords: X-linked Agammaglobulinemia; XLA; Neutropenia; Bruton’s Tyrosine Kinase; BTK; Immunodeficiency

Abstract

Objectives: X-linked Agammaglobulinemia (XLA) is a primary immunodeficiency disease, characterized by severe hypogammaglobulinemia and the low numbers of peripheral B cells. Neutropenia is a rare complication among the XLA patients, which may lead to a higher rate of infections and morbidity. The aim of the authors is to assess the correctness of this issue.

Methods: In this study, we compared demographic, clinical and laboratorial data between two groups of XLA patients, with and without neutropenia.

Results: Frequency of neutropenia was 15% in our population. Infectious complications were the most prevalent clinical manifestations, regardless of the presence of neutropenia. However, Lymphoproliferative complication was significantly higher in the neutropenic patients (p = 0.001). No significant difference in mortality rate was observed between the groups.

Conclusion: Neutropenia is a rare complication among the XLA patients, and significantly decreases the mean age of XLA diagnosis in the patients. But it is not related to the higher frequency of infectious diseases in the neutropenic patients compared to non-neutropenic ones.