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    Poster Networking

    Hemophilia and Rare Bleeding Disorders

    Poster Networking Session

    PB1300 - Intramural Hematoma of Gastrointestinal Tract in People With Hemophilia: A Pooled Study

    Tuesday, June 27, 2023
    18:30 – 19:30 ET
    Room: Exhibition

      Presenting Author(s)

    • Chia-Yau Chang, assistant professor

      Doctor
      1. Department of Pediatrics, School of Medicine, College of Medicine, Taipei Medical University; 2. Division of Pediatric Hematology and Oncology, Department of Pediatrics, Taipei Medical University Hospital, Taipei, Taiwan.
      Taipei, Taipei, Taiwan (Republic of China)

      • Co-Author(s)

    • WT

      Wei-Jung Teng

      Medical student
      School of Medicine, College of Medicine, Taipei Medical University, Taipei, Taiwan
      Taipei, Taipei, Taiwan (Republic of China)

    • CK

      Ching-Huei Kung

      Attending physician
      Department of Medical Imaging, Taipei Medical University Hospital, Taipei, Taiwan
      Taipei, Taipei, Taiwan (Republic of China)

    • MC

      Mei-Mei Cheng

      Attending physician
      Department of Pediatric Gastroenterology, Department of Pediatrics, Cheng-Hsin General Hospital, Taipei, Taiwan
      Taipei, Taipei, Taiwan (Republic of China)

    • JT

      Jia-Ruey Tsai

      Attending physician
      Division of Hematology/Oncology, Department of Internal Medicine, Taipei Medical University Hospital, Taipei, Taiwan
      Taipei, Taipei, Taiwan (Republic of China)

    Background: Intramural hematoma (IMH) of gastrointestinal tract is a quite rare bleeding condition in patients with hemophilia (PWH), and can be life-threatening. It lacked relatively whole analysis.

    Aims: We aimed to report our patient with IMH and conduct a pooled analysis with the literature.

    Methods: This pooled analysis included PWH with IMH treated at our institution and from other studies who were retrieved by using the keywords from the database of PubMed, Google Scholar, and Embase, covering the period from 1956 until October, 2022. The relevant data were collected for analysis.

    Results: One 48-year-old adult with severe-type hemophilia A receiving on-demand therapy in our hospital had experienced 2 episodes of spontaneous IMH within 3 months. After that, he received rFVIIIFc prophylaxis 60-65iu/kg/dose once weekly and had no more recurrence >3 years. Totally 83 patients, including our case, from 74 published studies were included. After excluding the missing data, there were 31 (38.75%) children and 49 (61.25%) adults. There were 62 hemophilia A and 4 hemophilia B, 4 acquired hemophilia. Among congenital hemophilia A patients, 23 were severe-type, 7 moderate-type, 5 mild-type, and 27 unknown-severity; they included 34 (70.8%) non-inhibitor patients and 14 (29.2%) inhibitor patients. Acute-onset abdominal pain was the most common symptoms. Small intestine was the most commonly involved(n=34, 41%), followed by colon(n=19, 23%), duodenum(n=15, 18%), stomach(n=10, 12%) and esophagus(n=5, 6%), with more details in Table 1. Mortality occurred in 7 of 30 (23.3%) children and 2 of 44 (4.5%) in adults, with overall mortality rate of 11.7 %. Compared to recovery group, mortality group had significantly higher ratio of pediatric patients and patients with duodenum or colon involved, showed as Table 2.

    Conclusion(s): The pooled study showed the most common involvement, mortality rate, and prognosis, providing a deeper insight and overall view into IMH in PWH. Prophylaxis therapy could decrease risk of recurrence.