The Evaluation of Neutropenia in X-linked Agammaglobulinemia Patients

Document Type : Original Article

Authors

1 Student Research Committee, School of Medicine, North Khorasan University of Medical Sciences, Bojnurd, Iran

2 Department of Pediatrics, North Khorasan University of Medical Sciences, Bojnurd, Iran

10.22034/igj.2021.264530.1057

Abstract

Objectives: X-linked agammaglobulinemia (XLA) is a primary immunodeficiency disease characterized by severe hypogammaglobulinemia and low numbers of peripheral B cells. Neutropenia is a rare complication among XLA patients which may leads to a higher rate of infections and morbidity. The aim of authors is to assess the correctness of this issue.
Methods: In this study, we compared demographic, clinical and laboratory 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 presence of neutropenia. However, Lymphoproliferative complication was significantly higher in neutropenic patients (p = 0.001). No significant difference in mortality was seen between the groups.
Conclusion: Neutropenia is a rare complication among XLA patients and significantly decreases the mean age of XLA diagnosis in the patients. But it is not related to higher frequency of infectious disease in neutropenic patients compared to non-neutropenic ones.

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