A Case With Wiskott-Aldrich Syndrome and Ascending Aorta Aneurysm.

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Barutcu A., Leblebisatan G., Leblebisatan S., Cil M., Sasmaz H. I., Demir F.

Journal of pediatric hematology/oncology, cilt.43, 2021 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 43
  • Basım Tarihi: 2021
  • Doi Numarası: 10.1097/mph.0000000000001932
  • Dergi Adı: Journal of pediatric hematology/oncology
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, CAB Abstracts, EMBASE, MEDLINE
  • Anahtar Kelimeler: aortic dilatation, immune thrombocytopenic purpura, resistant thrombocytopenia, Wiskott-Aldrich syndrome, RESECTION
  • Çukurova Üniversitesi Adresli: Evet

Özet

Wiskott-Aldrich syndrome (WAS) is a rare X-linked primary immunodeficiency disorder that is characterized by a triad of microthrombocytopenia, severe immunodeficiency, and eczema. We report the case of a 7-year-old male patient with chronic thrombocytopenia that was diagnosed as WAS after dilatation of the ascending aorta was noticed. WAS is rare, and it is a disease that requires high suspicion for diagnosis. We recommend periodic echocardiography and magnetic resonance imaging examinations to evaluate aortic aneurysms in children with WAS and that surgical intervention should not be delayed when aneurysm is detected.