Intest Res.  2020 Oct;18(4):469-475. 10.5217/ir.2019.09141.

Long-term outcome after surgery in a patient with intestinal Behçet’s disease complicated by myelodysplastic syndrome and trisomy 8

Affiliations
  • 1First Department of Internal Medicine, Faculty of Medicine, University of Yamanashi, Yamanashi, Japan
  • 2First Department of Surgery, Faculty of Medicine, University of Yamanashi, Yamanashi, Japan

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.

Keyword

Behcet syndrome; Myelodysplastic syndrome; Trisomy 8

Figure

  • Fig. 1. Endoscopic images. (A-D) Photographs of multiple punched-out ulcers in the ileocecum.

  • Fig. 2. Pathological findings. Histological image of biopsy specimens from the margin of the ileocecal ulcer show no malignant findings or nonspecific inflammatory changes at ×10 magnification of the objective lens (A) and ×4 magnification (B); the magnification of the objective lens is not identified because camera systems have become an integral part of the microscope (H&E). (C) Ileocecal resection with ileostomy reveals penetration of the ileum, as well as ileal ulcers.

  • Fig. 3. Therapy flowchart. First, intensive treatment with 5-aminosalicylic acid (5-ASA), colchicine was ineffective, so we decided to administer prednisolone (PSL) on readmission. Next, mercaptopurine was administered for intestinal Behçet’s disease, but this treatment was discontinued due to liver damage. PSL was effective and tapered slowly over several weeks, but his abdominal pain and fever steadily worsened and computed tomography revealed perforation of the ileum. Therefore, ileocecal resection with ileostomy was performed. The 0 day refers to the day of readmission, 1 day refers to the day of PSL administration. The 58 days refers to the day of recurrence, 77 days refers to the day of operation. (A) At diagnosis (1.5 years ago), (B) on admission (1 year ago), and (C) at recurrence (58 days). ADA, adalimumab; CRP, C-reactive protein.

  • Fig. 4. Abdominal computed tomography (CT) found wall thickening of the ileocecum, disproportionation fat stranding, and ileal perforation. (A) CT on admission, (B) CT at the recrudescent time. The arrows indicate ileal perforation.

  • Fig. 5. The endoscopic image of about 5 years after the anastomosis.


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