HOMEPRODUCTSCOMPANYCONTACTFAQResearchDictionaryPharmaSign Up FREE or Login

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

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.
AuthorsKostas N Syrigos, Nektaria Makrilia, Jeffrey Neidhart, Michael Moutsos, Sotirios Tsimpoukis, Maria Kiagia, Muhammad W Saif
JournalItalian journal of pediatrics (Ital J Pediatr) Vol. 37 Pg. 42 (Sep 10 2011) ISSN: 1824-7288 [Electronic] England
PMID21906397 (Publication Type: Journal Article)
Topics
  • Bone Marrow Transplantation (methods)
  • Follow-Up Studies
  • Humans
  • Male
  • Patient Compliance
  • Patient Education as Topic
  • Postoperative Care (methods)
  • Splenectomy (methods)
  • Time Factors
  • Wiskott-Aldrich Syndrome (surgery)
  • Young Adult

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.
Realize the full power of the drug-disease research graph!


Choose Username:
Email:
Password:
Verify Password:
Enter Code Shown: