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Long-term outcome after surgery in a patient with intestinal Behçet’s disease complicated by myelodysplastic syndrome and trisomy 8
- Source :
- Intestinal Research, Vol 18, Iss 4, Pp 469-475 (2020), Intestinal Research
- Publication Year :
- 2020
- Publisher :
- Korean Association for the Study of Intestinal Diseases, 2020.
-
Abstract
- Behçet’s disease (BD) is a multisystem inflammatory disease of unknown origin. Rarely, BD occurs together with myelodysplastic syndrome (MDS). Interestingly, it is speculated that these are not simple coexistence but that the etiology of intestinal BD is at least partly derived from MDS itself. Furthermore, there is a relationship between MDS in patients with intestinal BD and trisomy 8. Immunosuppressive agents alone are insufficient to control MDS-associated BD, and many of these patients die of infection or hemorrhage. Surgery is considered for intestinal BD patients who are unresponsive to medical treatment or those with bowel complications such as perforation or persistent bleeding. We report a case of intestinal BD associated with MDS and trisomy 8. The patient was unresponsive to oral steroids and immunosuppressive treatment; the patient improved by surgical repair of a bowel perforation. Five years after the surgery, the patient is free of recurrence and not on medication. Our experience suggests that surgery may provide an effective therapeutic option for the treatment of MDS-related BD.
- Subjects :
- medicine.medical_specialty
Perforation (oil well)
lcsh:Medicine
Case Report
Behcet's disease
Bowel perforation
Disease
behcet syndrome
Trisomy 8
03 medical and health sciences
0302 clinical medicine
trisomy 8
hemic and lymphatic diseases
Medicine
In patient
030212 general & internal medicine
lcsh:RC799-869
Medical treatment
business.industry
lcsh:R
Gastroenterology
medicine.disease
Surgery
myelodysplastic syndrome
Etiology
030211 gastroenterology & hepatology
lcsh:Diseases of the digestive system. Gastroenterology
business
Subjects
Details
- Language :
- English
- ISSN :
- 22881956 and 15989100
- Volume :
- 18
- Issue :
- 4
- Database :
- OpenAIRE
- Journal :
- Intestinal Research
- Accession number :
- edsair.doi.dedup.....d2ce3538a540356f1f9e4f378712822b