Optimal Treatment Approaches to Intestinal Behçet's Disease Complicated by Myelodysplastic Syndrome: The KASID and KSBD Multicenter Study
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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.
Abstract
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.
참고문헌 (44)
- Sakane T, "Behçet’s disease", N Engl J Med, 341, pp.1284~1291 (1999)
- Kaklamani VG, "Behçet’s disease", Semin Arthritis Rheum, 27, pp.197~217 (1998)
- Ebert EC, "Gastrointestinal manifestations of Behçet’s disease", Dig Dis Sci, 54, pp.201~207 (2009)
- Skef W, "Gastrointestinal Behçet’s disease : a review", World J Gastroenterol, 21, pp.3801~3812 (2015)
- Lee HJ, "Optimal diagnosis and disease activity monitoring of intestinal Behçet’s disease", Intest Res, 15, pp.311~317 (2017)
- Kobayashi K, "Development of consensus statements for the diagnosis and management of intestinal Behçet’s disease using a modified Delphi approach", J Gastroenterol, 42, pp.737~745 (2007)
- Hisamatsu T, "The 2nd edition of consensus statements for the diagnosis and management of intestinal Behçet’s disease : indication of anti-TNFα monoclonal antibodies", J Gastroenterol, 49, pp.156~162 (2014)
- Hisamatsu T, "Treatment and outcomes : medical and surgical treatment for intestinal Behçet’s disease", Intest Res, 15, pp.318~327 (2017)
- Na SY, "Korean clinical practice guidelines on biologics and small molecules for moderate-to-severe ulcerative colitis", Intest Res, 21, pp.61~87 (2023)
- Koh SJ, "Korean clinical practice guidelines on biologics for moderate to severe Crohn’s disease", Intest Res, 21, pp.43~60 (2023)
- Singh A, "Use of thiopurines in inflammatory bowel disease : an update", Intest Res, 20, pp.11~30 (2022)
- Adès L, "Myelodysplastic syndromes", Lancet, 383, pp.2239~2252 (2014)
- Bejar R, "Clinical effect of point mutations in myelodysplastic syndromes", N Engl J Med, 364, pp.2496~2506 (2011)
- Gañán-Gómez I, "Deregulation of innate immune and inflammatory signaling in myelodysplastic syndromes", Leukemia, 29, pp.1458~1469 (2015)
- Kristinsson SY, "Chronic immune stimulation might act as a trigger for the development of acute myeloid leukemia or myelodysplastic syndromes", J Clin Oncol, 29, pp.2897~2903 (2011)
- de Hollanda A, "Systemic and immune manifestations in myelodysplasia : a multicenter retrospective study", Arthritis Care Res(Hoboken), 63, pp.1188~1194 (2011)
- Komrokji RS, "Autoimmune diseases and myelodysplastic syndromes", Am J Hematol, 91, pp.E280~E283 (2016)
- Mekinian A, "Systemic inflammatory and autoimmune manifestations associated with myelodysplastic syndromes and chronic myelomonocytic leukaemia : a French multicentre retrospective study", Rheumatology(Oxford), 55, pp.291~300 (2016)
- Montoro J, "Autoimmune disorders are common in myelodysplastic syndrome patients and confer an adverse impact on outcomes", Ann Hematol, 97, pp.1349~1356 (2018)
- Greenberg PL, "Revised international prognostic scoring system for myelodysplastic syndromes", Blood, 120, pp.2454~2465 (2012)
- Ohno E, "Behçet’s disease associated with myelodysplastic syndromes. A case report and a review of the literature", Cancer, 79, pp.262~268 (1997)
- Kawabata H, "Myelodysplastic syndrome complicated with inflammatory intestinal ulcers : significance of trisomy 8", Intern Med, 45, pp.1309~1314 (2006)
- Nonami A, "Successful treatment of myelodysplastic syndrome(MDS)-related intestinal Behçet’s disease by up-front cord blood transplantation", Intern Med, 46, pp.1753~1756 (2007)
- Ahn JK, "Behcet’s disease associated with bone marrow failure in Korean patients : clinical characteristics and the association of intestinal ulceration and trisomy 8", Rheumatology(Oxford), 47, pp.1228~1230 (2008)
- Toyonaga T, "Refractoriness of intestinal Behçet’s disease with myelodysplastic syndrome involving trisomy 8 to medical therapies-our case experience and review of the literature", Digestion, 88, pp.217~221 (2013)
- Asano T, "Intestinal Behçet disease associated with myelodysplastic syndrome accompanying trisomy 8 successfully treated with abdominal surgery followed by hematopoietic stem cell transplantation : a case report", Medicine(Baltimore), 98, pp.e17979~ (2019)
- Yilmaz U, "How to treat myelodysplastic syndrome with clinical features resembling Behçet syndrome : a case-based systematic review", Ann Hematol, 99, pp.1193~1203 (2020)
- Kawano S, "Clinical features of intestinal Behçet’s disease associated with myelodysplastic syndrome and trisomy 8", Acta Med Okayama, 69, pp.365~369 (2015)
- Wesner N, "Gastrointestinal Behcet’s-like disease with myelodysplastic neoplasms with trisomy 8 : a French case series and literature review", Leuk Lymphoma, 60, pp.1782~1788 (2019)
- Shen Y, "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", Biomed Res Int, 2018, pp.8535091~ (2018)
- Mori Y, "Long-term outcome after surgery in a patient with intestinal Behçet’s disease complicated by myelodysplastic syndrome and trisomy 8", Intest Res, 18, pp.469~475 (2020)
- Watanabe K, "Evidence-based diagnosis and clinical practice guidelines for intestinal Behçet’s disease 2020 edited by intractable diseases, the health and labour sciences research grants", J Gastroenterol, 55, pp.679~700 (2020)
- Bernasconi P, "Incidence and prognostic significance of karyotype abnormalities in de novo primary myelodysplastic syndromes : a study on 331 patients from a single institution", Leukemia, 19, pp.1424~1431 (2005)
- Haase D, "Cytogenetic features in myelodysplastic syndromes", Ann Hematol, 87, pp.515~526 (2008)
- Esatoglu SN, "A reappraisal of the association between Behçet’s disease, myelodysplastic syndrome and the presence of trisomy 8 : a systematic literature review", Clin Exp Rheumatol, 33(6 Suppl 94), pp.S145~S151 (2015)
- Tunc R, "Target organ associations in Turkish patients with Behçet’s disease : a cross sectional study by exploratory factor analysis", J Rheumatol, 29, pp.2393~2396 (2002)
- Shimizu T, "Behçet disease(Behçet syndrome)", Semin Arthritis Rheum, 8, pp.223~260 (1979)
- Chen G, "Distinctive gene expression profiles of CD34 cells from patients with myelodysplastic syndrome characterized by specific chromosomal abnormalities", Blood, 104, pp.4210~4218 (2004)
- Hasegawa H, "Behçet’s disease associated with myelodysplastic syndrome with elevated levels of inflammatory cytokines", Mod Rheumatol, 13, pp.350~355 (2003)
- Suzuki Y, "Long-term safety and effectiveness of adalimumab in 462 patients with intestinal Behçet’s disease : results from a large realworld observational study", Intest Res, 19, pp.301~312 (2021)
- Campioni D, "Evidence for a role of TNF-related apoptosis-inducing ligand(TRAIL)in the anemia of myelodysplastic syndromes", Am J Pathol, 166, pp.557~563 (2005)
- Düzgün N, "Cytokine inhibitors : soluble tumor necrosis factor receptor 1 and interleukin-1 receptor antagonist in Behçet’s disease", Rheumatol Int, 25, pp.1~5 (2005)
- Rossi G, "Autologous hematopoietic stem cell transplantation for severe", refractory intestinal Behcet disease, 2004, pp.103, 748~750
- Giannouli S, "Autoimmune phenomena in myelodysplastic syndromes : a 4-yr prospective study", Rheumatology(Oxford), 43, pp.626~632 (2004)