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

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.

Long‐term clinical outcomes of intestinal Behçet's disease: A 30‐year cohort study at a tertiary hospital in South Korea

“…Patients with intestinal BD presented with myelodysplastic syndrome and aplastic anemia in 1.5% and 1.0% of cases, respectively, which is a prominent hematologic finding of intestinal BD. [20][21][22] The patients with intestinal BD had colon and rectal cancer in 0.1% of cases each, which was similar to that in the general population, unlike CD. 23,24 The annual diagnosis of intestinal BD increased from 1997 to 2006, peaked between 2007 and 2011, and decreased from 2012 to 2021 in our hospital.…”

“…Contrary to other clinical outcomes, the mortality rate of intestinal BD appears to be high, which might have been influenced by the older age of patients with intestinal BD and the presence of comorbidities, including hematologic disorders. Patients with intestinal BD presented with myelodysplastic syndrome and aplastic anemia in 1.5% and 1.0% of cases, respectively, which is a prominent hematologic finding of intestinal BD 20–22 . The patients with intestinal BD had colon and rectal cancer in 0.1% of cases each, which was similar to that in the general population, unlike CD 23,24 …”

Clinical Course of Patients with Intestinal Behçet’s Disease According to Consensus-Based Diagnostic Categories

“…[17][18][19] Patients with Behçet's disease and bone marrow failure showed more frequent intestinal involvement and refractoriness to steroids and immunomodula-tors 16 and the proportion of trisomy 8 was reported in up to 64%-86% of patients with Behçet's disease and bone marrow failure. [20][21][22] In our study, accompanying hematologic disorders at the time of diagnosis were associated with a higher risk of major surgery or admission related to iBD (HR, 2.91; 95% CI, 1.18 to 7.13). Of the 118 patients with definite, probable, and suspected iBD, hematologic disorders were present in 20 (16.9%) and there were seven patients with trisomy 8 among the 15 patients with bone marrow failure (46.7%).…”

Optimal Treatment Approaches to Intestinal Behçet's Disease Complicated by Myelodysplastic Syndrome: The KASID and KSBD Multicenter Study

“… 29 30 Particularly, patients with intestinal BD complicated by MDS (intestinal BD–MDS) with cytogenetic aberration trisomy 8 often have refractoriness to conventional medical treatments and poor prognoses. 25 27 31 Immunomodulators and anti-TNFα agents had no beneficial effects on patients with intestinal BD–MDS compared to those with intestinal BD without MDS and improved through treatment targeted to MDS. 24 25 26 27 However, studies on Korean patients with intestinal BD–MDS are rare, and no established therapeutic guidelines exist.…”