Recurrent spontaneous splenic rupture in a patient with congenital factor XIII deficiency

Amir H. Shariff, Muhammad Waqas, Basit Salam, Muhammad Arshad, Salman N. Adil

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2 Citations (Scopus)


We describe an unusual presentation of factor XIII (FXIII) deficiency in a 17-year-old boy who was diagnosed with this congenital deficiency at the age of 18 months. He had a history of spontaneous splenic rupture 8 years ago, which was managed conservatively. He now presented with sudden severe abdominal and left shoulder pain for 1 day, with no history of antecedent trauma. He was in shock, and examination revealed diffuse peritonitis. A computed tomography scan showed a grade IV splenic laceration. He was taken as an emergency to the operating room where he was found to have a shattered spleen, and a splenectomy was performed. He received cryoprecipitate transfusions perioperatively. After an uneventful recovery, the patient was discharged. To the best of our knowledge, this is the first described case of a recurrent splenic rupture in a patient with FXIII deficiency.

Original languageEnglish
Pages (from-to)471-473
Number of pages3
JournalJournal of Pediatric Hematology/Oncology
Issue number6
Publication statusPublished - Aug 2014


  • factor XIII
  • factor XIII deficiency
  • splenectomy
  • spontaneous splenic rupture


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