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J Pediatr Hematol Oncol. 2014 Aug;36(6):471-3. doi: 10.1097/MPH.0000000000000088.

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

Author information

1
Departments of *Surgery †Radiology ‡Oncology, The Aga Khan University, Karachi, Pakistan.

Abstract

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.

PMID:
24351968
DOI:
10.1097/MPH.0000000000000088
[Indexed for MEDLINE]

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