title: 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.
authors: Oncology Unit, 3rd Department of Medicine, Sotiria General Hospital, Athens School of Medicine, Greece. knsyrigos@usa.net
authors: Kostas N
authors: KN
authors: Syrigos
authors: Nektaria
authors: N
authors: Makrilia
authors: Jeffrey
authors: J
authors: Neidhart
authors: Michael
authors: M
authors: Moutsos
authors: Sotirios
authors: S
authors: Tsimpoukis
authors: Maria
authors: M
authors: Kiagia
authors: Muhammad W
authors: MW
authors: Saif
mesh_terms: D016026:Bone Marrow Transplantation / Q000379:methods*; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D010349:Patient Compliance; D010353:Patient Education as Topic; D011182:Postoperative Care / Q000379:methods*; D013156:Splenectomy / Q000379:methods*; D013997:Time Factors; D014923:Wiskott-Aldrich Syndrome / Q000601:surgery*; D055815:Young Adult