Prolonged survival after splenectomy in Wiskott-Aldrich syndrome: a case report
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.
- Prolonged survival after splenectomy in Wiskott-Aldrich syndrome: a case report
- Open Access
- Available under Open Access This content is freely available online to anyone, anywhere at any time.
Italian Journal of Pediatrics
- Online Date
- September 2011
- Online ISSN
- BioMed Central
- Additional Links
- Wiskott-Aldrich syndrome
- Author Affiliations
- 1. Oncology Unit, 3rd Department of Medicine, Sotiria General Hospital, Athens School of Medicine, Kragujevac, Greece
- 2. San Juan Oncology, New Mexico, USA
- 3. Division of Hematology and Oncology, Department of Medicine, Columbia University, Kragujevac, NY, USA