Behçet's disease (BD) is a disorder characterized by systemic inflammation of multiple organs, including the intestines. Several studies have reported a relationship between myelodysplastic syndrome and BD, and trisomy 8 was frequently seen, especially in intestinal BD. However, the association of BD with primary myelofibrosis (PMF) has not been well documented. A 58-year-old Japanese female was diagnosed with PMF in 2014. The symptoms of PMF resolved with ruxolitinib. However, she developed fever and intestinal perforation due to multiple ulcers in the terminal ileum in 2017. Intestinal perforation recurred 1 month later, and the dose of ruxolitinib was tapered. After discontinuation of ruxolitinib, she presented with recurrent oral aphthous ulcers and uveitis. Subsequently, intestinal perforation recurred, and she was diagnosed with intestinal BD. Trisomy 8 was identified in her peripheral blood. She underwent steroid therapy, azathioprine, and infliximab. This case suggests relationships between PMF, trisomy 8, and BD.

Download full-text PDF

Source
http://dx.doi.org/10.1159/000501019DOI Listing

Publication Analysis

Top Keywords

intestinal perforation
12
behçet's disease
8
primary myelofibrosis
8
perforation recurred
8
intestinal
6
intestinal behçet's
4
disease primary
4
myelofibrosis involving
4
trisomy
4
involving trisomy
4

Similar Publications

Background: Gastrointestinal perforation (GIP) is a life-threatening condition that necessitates immediate surgical intervention. This study aims to identify prognostic factors in patients with GIP treated within a standardized acute care surgery (ACS) framework.

Materials And Methods: This single center retrospective cohort study analyzed patients diagnosed with GIP who underwent emergent surgery and were admitted to the intensive care unit between January 2013 and March 2023.

View Article and Find Full Text PDF

Through-the-scope tack application for defect closure following endoscopic submucosal dissection.

Tech Coloproctol

December 2024

Department of Colorectal Surgery, Digestive Disease and Surgery Institute, Cleveland Clinic, 9500 Euclid Avenue A-30, Cleveland, OH, 44195, USA.

Effective closure of both muscular and mucosal defects after endoscopic submucosal dissection (ESD) remains a technical challenge. Failure to adequately address such defects may increase the risk of delayed perforation. In this video case report, we demonstrate the application of the through-the-scope (TTS) tack system for defect closure following ESD in a patient with a cecal lesion.

View Article and Find Full Text PDF

Background: This study aimed to evaluate the risk factors associated with rectal perforation during various surgical interventions for presacral cysts.

Methods: This retrospective study included 73 participants from 2013 to 2023 who met the inclusion criteria. Participants underwent surgical treatments through transabdominal, perineal, or combined approaches.

View Article and Find Full Text PDF

Although rearrangement of the MYC oncogene (MYC-R) is frequently observed in aggressive B-cell lymphomas, it is extremely rare in T-cell malignancies. A 64-year-old man who had been under observation for several years because of asymptomatic pulmonary extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALToma) was admitted to our hospital because of poor general condition and hypotension. Blood tests revealed thrombocytopenia and elevated serum lactate dehydrogenase levels, whereas computed tomography revealed systemic lymphadenopathy and splenomegaly.

View Article and Find Full Text PDF

Necrotising fasciitis (NF) is a rare surgical emergency characterised by soft tissue necrosis and systemic compromise. Typically it originates following adjacent skin and soft tissue insult; however, our unusual case required a high index of clinical suspicion to avert potential mortality. A man in his 60s with diabetes mellitus presented with 2 weeks of knee pain, swelling and necrotic skin on the posterior calf.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!