Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2023 Apr 24;12(9):3093.
doi: 10.3390/jcm12093093.

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

Wei-Jung Teng  1 , Ching-Huei Kung  2 , Mei-Mei Cheng  3 , Jia-Ruey Tsai  4   5 , Chia-Yau Chang  5   6   7
Affiliations
Review

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

Wei-Jung Teng et al. J Clin Med. .

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.

Keywords: clotting factor concentrates; conservative treatment; factor VIII inhibitor; hemophilia; intramural hematoma.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
First episode of intramural hematoma of jejunum. (a) Axial non-contrast CT showed marked segmental wall thickening of the proximal jejunum with diffuse hyperdensity (arrow), (b) axial non-contrast CT showed perisplenic hemoperitoneum (arrow), (c) coronal reformatted contrast-enhanced CT showed contrast enhancement of the inner mucosa, but there was no contrast enhancement of the thickened wall of the corresponding proximal jejunum, which indicated the presence of an intramural hematoma (arrow).
Figure 2
Figure 2
Second episode of intramural hematoma of jejunum. (a) Axial non-contrast CT showed segmental thickening of the midjejunum with diffuse hyperdensity, indicating intramural hematoma (arrow), (b) coronal reformatted contrast-enhanced CT showed intramural hematoma (*) of the midjejunum and marked dilatation of the proximal jejunum, indicating mechanical ileus (arrow).
Figure 3
Figure 3
Flowchart of paper selection.
See this image and copyright information in PMC

References

    1. Salma F., Soukaina W. Conservative Management of a Jejuno-Jejunal Intussusception in a Patient with Severe Haemophilia A. Curr. Health Sci. J. 2021;47:462–465. doi: 10.12865/chsj.47.03.20. - DOI - PMC - PubMed
    1. Delibegovic M., Alispahic A., Gazija J., Mehmedovic Z., Mehmedovic M. Intramural Haemorrhage and Haematoma as the Cause of Ileus of the Small Intestine in a Haemophiliac. Med. Arch. 2015;69:206–207. doi: 10.5455/medarh.2015.69.206-207. - DOI - PMC - PubMed
    1. Ramadan K.M., Lowry J.P., Wilkinson A., McNulty O., McMullin M.F., Jones F.G. Acute intestinal obstruction due to intramural haemorrhage in small intestine in a patient with severe haemophilia A and inhibitor. Eur. J. Haematol. 2005;75:164–166. doi: 10.1111/j.1600-0609.2005.00445.x. - DOI - PubMed
    1. Prieto H.M.M., Perez C.P., Bermejo J.M.B. Intramural Hematoma of the Small Intestine in a Patient with Severe Hemophilia A. Case Report and Review of the Literature. J. Gastrointest. Dig. Syst. 2015;5:6. doi: 10.4172/2161-069X.1000363. - DOI
    1. Katsumi A., Matsushita T., Hirashima K., Iwasaki T., Adachi T., Yamamoto K., Kojima T., Takamatsu J., Saito H., Naoe T. Recurrent intramural hematoma of the small intestine in a severe hemophilia A patient with a high titer of factor VIII inhibitor: A case report and review of the literature. Int. J. Hematol. 2006;84:166–169. doi: 10.1532/IJH97.06053. - DOI - PubMed

LinkOut - more resources