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Purpose: Studies on intestinal Behçet’s disease (BD) complicated by myelodysplastic syndrome (MDS) are rare, and no established therapeutic guidelines exist. This study aimed to evaluate the clinical presentation and outcomes of patients with intestinal BD complicated by MDS (intestinal BD–MDS) and suggest a treatment strategy.

Materials and Methods: Data from patients with intestinal BD–MDS from four referral centers in Korea who were diagnosed between December 2000 and December 2022 were retrospectively analyzed. Clinical features and prognosis of intestinal BD–MDS compared with age-, sex-matched intestinal BD without MDS were investigated.

Results: Thirty-five patients with intestinal BD–MDS were included, and 24 (70.6%) had trisomy 8. Among the 35 patients, 23 (65.7%) were female, and the median age at diagnosis for intestinal BD was 46.0 years (range, 37.0–56.0 years). Medical treatments only benefited eight of the 32 patients, and half of the patients underwent surgery due to complications. Compared to 70 matched patients with intestinal BD alone, patients with intestinal BD–MDS underwent surgery more frequently (51.4% vs. 24.3%; p=0.010), showed a poorer response to medical and/or surgical treatment (75.0% vs. 11.4%; p〈0.001), and had a higher mortality (28.6% vs. 0%; p〈0.001). Seven out of 35 patients with intestinal BD–MDS underwent hematopoietic stem cell transplantation (HSCT), and four out of the seven patients had a poor response to medical treatment prior to HSCT, resulting in complete remission of both diseases.

Conclusion: Patients with intestinal BD–MDS frequently have refractory diseases with high mortalities. HSCT can be an effective treatment modality for medically refractory patients with intestinal BD–MDS.

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Optimal treatment approaches to intestinal Behçet's disease complicated by myelodysplastic syndrome : the KASID and KSBD multicenter study Jung-Bin Park, So Jung Han, Seung Bum Lee, Dong Hyun Kim, Jae Hee Cheon, Sung Wook Hwang, Byong Duk Ye, Suk-Kyun Yang, Soo Jung Park, Sang Hyoung Park p. 265-275, [1]

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참고문헌 (44건) : 자료제공( 네이버학술정보 )

참고문헌 목록에 대한 테이블로 번호, 참고문헌, 국회도서관 소장유무로 구성되어 있습니다.
번호 참고문헌 국회도서관 소장유무
1 Beh�et's disease associated with myelodysplastic syndromes 미소장
2 Systemic inflammatory and autoimmune manifestations associated with myelodysplastic syndromes and chronic myelomonocytic leukaemia: a French multicentre retrospective study 미소장
3 Cytokine inhibitors: soluble tumor necrosis factor receptor 1 and interleukin-1 receptor antagonist in Behçet’s disease 미소장
4 Successful Treatment of Myelodysplastic Syndrome (MDS)-related intestinal Behcet's Disease by Up-front Cord Blood Transplantation 미소장
5 Gastrointestinal Behçet's disease: A review 미소장
6 Myelodysplastic Syndrome Complicated with Inflammatory Intestinal Ulcers: Significance of Trisomy 8 미소장
7 Cytogenetic features in myelodysplastic syndromes 미소장
8 Development of consensus statements for the diagnosis and management of intestinal Behçet's disease using a modified Delphi approach 미소장
9 Behçet disease (Behçet syndrome) 미소장
10 Deregulation of innate immune and inflammatory signaling in myelodysplastic syndromes 미소장
11 The 2nd edition of consensus statements for the diagnosis and management of intestinal Behçet’s disease: indication of anti-TNFα monoclonal antibodies 미소장
12 Incidence and prognostic significance of karyotype abnormalities in de novo primary myelodysplastic syndromes: a study on 331 patients from a single institution 미소장
13 Distinctive gene expression profiles of CD34 cells from patients with myelodysplastic syndrome characterized by specific chromosomal abnormalities 미소장
14 Systemic and immune manifestations in myelodysplasia: A multicenter retrospective study 미소장
15 Gastrointestinal Manifestations of Behçet’s Disease 미소장
16 Autologous hematopoietic stem cell transplantation for severe/refractory intestinal Behcet disease 미소장
17 Clinical Effect of Point Mutations in Myelodysplastic Syndromes 미소장
18 Refractoriness of Intestinal Behçet's Disease with Myelodysplastic Syndrome Involving Trisomy 8 to Medical Therapies - Our Case Experience and Review of the Literature 미소장
19 Evidence for a Role of TNF-Related Apoptosis-Inducing Ligand (TRAIL) in the Anemia of Myelodysplastic Syndromes 미소장
20 Chronic Immune Stimulation Might Act As a Trigger for the Development of Acute Myeloid Leukemia or Myelodysplastic Syndromes 미소장
21 Autoimmune phenomena in myelodysplastic syndromes: a 4-yr prospective study 미소장
22 Behcet's disease associated with bone marrow failure in Korean patients: clinical characteristics and the association of intestinal ulceration and trisomy 8 미소장
23 Behçet’s disease associated with myelodysplastic syndrome with elevated levels of inflammatory cytokines 미소장
24 Clinical Features of Intestinal Behçet's Disease Associated with Myelodysplastic Syndrome and Trisomy 8. 미소장
25 Autoimmune diseases and myelodysplastic syndromes 미소장
26 Target organ associations in Turkish patients with Behçet's disease: a cross sectional study by exploratory factor analysis. 미소장
27 A reappraisal of the association between Behçet's disease, myelodysplastic syndrome and the presence of trisomy 8: a systematic literature review. 미소장
28 Revised International Prognostic Scoring System for Myelodysplastic Syndromes 미소장
29 Treatment and outcomes: medical and surgical treatment for intestinal Behçet's disease 미소장
30 Optimal diagnosis and disease activity monitoring of intestinal Behçet's disease 미소장
31 Autoimmune disorders are common in myelodysplastic syndrome patients and confer an adverse impact on outcomes 미소장
32 High Incidence of Gastrointestinal Ulceration and Cytogenetic Aberration of Trisomy 8 as Typical Features of Behçet’s Disease Associated with Myelodysplastic Syndrome: A Series of 16 Consecutive Chinese Patients from the Shanghai Behçet’s Disease Database and Comparison with the Literature 미소장
33 Gastrointestinal Behcet’s-like disease with myelodysplastic neoplasms with trisomy 8: a French case series and literature review 미소장
34 Intestinal Behçet disease associated with myelodysplastic syndrome accompanying trisomy 8 successfully treated with abdominal surgery followed by hematopoietic stem cell transplantation 미소장
35 How to treat myelodysplastic syndrome with clinical features resembling Behçet syndrome: a case-based systematic review 미소장
36 Long-term outcome after surgery in a patient with intestinal Behçet’s disease complicated by myelodysplastic syndrome and trisomy 8 미소장
37 Evidence-based diagnosis and clinical practice guidelines for intestinal Behçet’s disease 2020 edited by Intractable Diseases, the Health and Labour Sciences Research Grants 미소장
38 Long-term safety and effectiveness of adalimumab in 462 patients with intestinal Behçet’s disease: results from a large real-world observational study 미소장
39 Use of thiopurines in inflammatory bowel disease: an update 미소장
40 Behçet's Disease 미소장
41 Behçet's Disease 미소장
42 Korean clinical practice guidelines on biologics and small molecules for moderate-to-severe ulcerative colitis 미소장
43 Myelodysplastic syndromes 미소장
44 Korean clinical practice guidelines on biologics for moderate to severe Crohn’s disease 미소장

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