Case report

Italian Journal of Pediatrics

, 37:42

Open Access This content is freely available online to anyone, anywhere at any time.

Prolonged survival after splenectomy in Wiskott-Aldrich syndrome: a case report

  • Kostas N SyrigosAffiliated withOncology Unit, 3rd Department of Medicine, Sotiria General Hospital, Athens School of Medicine Email author 
  • , Nektaria MakriliaAffiliated withOncology Unit, 3rd Department of Medicine, Sotiria General Hospital, Athens School of Medicine
  • , Jeffrey NeidhartAffiliated withSan Juan Oncology
  • , Michael MoutsosAffiliated withOncology Unit, 3rd Department of Medicine, Sotiria General Hospital, Athens School of Medicine
  • , Sotirios TsimpoukisAffiliated withOncology Unit, 3rd Department of Medicine, Sotiria General Hospital, Athens School of Medicine
  • , Maria KiagiaAffiliated withOncology Unit, 3rd Department of Medicine, Sotiria General Hospital, Athens School of Medicine
  • , Muhammad W SaifAffiliated withDivision of Hematology and Oncology, Department of Medicine, Columbia University

Abstract

Wiskott-Aldrich syndrome is a rare X-linked immunodeficiency disorder that is characterized by a variable clinical phenotype. Matched donor bone marrow transplantation is currently the only curative therapeutic option. We present the case of a 24-year-old male who was diagnosed at the age of seven with Wiskott-Aldrich syndrome. He did not respond to intravenous gammaglobulin and he experienced recurrent pulmonary infections despite prophylactic antibiotics. The patient had no matched donor. At the age of nine, he was submitted to splenectomy and his platelet count was normalized. Fifteen years later, the patient remains asymptomatic with a normal platelet count. He is still receiving prophylactic antibiotics and no bleeding episodes or septic complications have been reported. This case demonstrates that splenectomy can represent a safe therapeutic option in selected WAS patients, provided that there is a tight follow-up program, patient education and adherence to guidelines regarding post-splenectomy prophylaxis.

Keywords

Wiskott-Aldrich syndrome thrombocytopenia splenectomy survival treatment