Intramural Hematoma of Gastrointestinal Tract in People with Hemophilia A and B

Wei-Jung Teng, Ching-Huei Kung, Mei-Mei Cheng, Jia-Ruey Tsai, Chia-Yau Chang

Research output: Contribution to journalReview articlepeer-review

2 Citations (Scopus)

Abstract

People with hemophilia (PWH), especially severe hemophilia, often experience bleeding episodes, which occur mostly at major joints. Intramural hematoma of the gastrointestinal (GI) tract is a rare, potentially life-threatening clinical bleeding manifestation in PWH. Prompt identification and timely administration of clotting factor concentrates are of utmost importance for effective management and optimal patient outcomes. In this report, we present the case of a 48-year-old male with severe hemophilia A. The patient developed a spontaneous intramural hematoma of the jejunum, leading to signs of acute abdomen, bloody stool, and paralytic ileus. Conservative management with factor VIII (FVIII) infusion was successfully administered. However, within a span of three months, the patient suffered from a recurrent episode of intramural hematoma, which was again effectively treated with conservative therapy. Subsequently, prophylactic FVIII therapy was administered to the patient, resulting in the absence of recurrence for over three years. Inspired by this case, we conducted a comprehensive review of the relevant literature and gathered data from 79 reported cases of intramural hematoma that were documented between the years 1956 and 2022. We classified these cases based on the site affected within the gastrointestinal (GI) tract (spread across five different locations) and proceeded to conduct a simple pooling analysis on the data collected, which subsequently revealed that the overall mortality rate of intramural hematoma in people with hemophilia (PWH) was found to be 12.2%, while children have a higher mortality rate (23.3%) than adults (4.9%). We hope this case report and literature review increase awareness of this rare bleeding manifestation in PWH, the effectiveness of conservative treatment, and the possibility of prophylaxis against recurrence.
Original languageEnglish
Article number3093
Pages (from-to)3093
JournalJournal of Clinical Medicine
Volume12
Issue number9
DOIs
Publication statusPublished - May 2023

Keywords

  • clotting factor concentrates
  • conservative treatment
  • factor VIII inhibitor
  • hemophilia
  • intramural hematoma

ASJC Scopus subject areas

  • General Medicine

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