title: X-linked agammaglobulinemia presenting with secondary hemophagocytic syndrome: a case report.
abstract: Introduction. Coincidence of X-linked agammaglobulinemia (XLA) and secondary hemophagocytic syndrome (sHS) is atypical. Both diseases are rare and pathogenesis of the latter one is not clearly known. Case Presentation. A 5-year-old boy was diagnosed both with XLA and sHS. However, in his history, he did not have severe and recurrent infections. Bruton tyrosine kinase (BTK) gene mutation was present (c.1581_1584delTTTG). To the best of the authors' knowledge, coincidence of XLA and sHS had not been reported in the literature before. Conclusion. Patients with XLA are extremely vulnerable to recurrent bacterial infections. The diagnosis of XLA with sHS at any time of life is both an interesting and challenging situation without history of recurrent bacterial infections.
authors: Department of Pediatrics, Izmir Tepecik Education and Research Hospital, Ministry of Health, 35100 Izmir, Turkey.
authors: Can
authors: C
authors: Ozturk
authors: Sumer
authors: S
authors: Sutcuoglu
authors: Berna
authors: B
authors: Atabay
authors: Afig
authors: A
authors: Berdeli