The Association Between Behcet's Disease, Myelodysplastic Syndrome and Trisomy 8

Won-Seok Lee¹, Yun Jung Choi², Myong-Joo Hong³, Chang-Hoon Lee⁴, Myeung Su Lee⁵, Young Sun Suh⁶, Sang-Il Lee⁷, Yong Gon Cho⁸ and Wan-Hee Yoo⁹, ¹Division of Rheumatology, Department of Internal Medicine, Chonbuk National University Medical School and Research Institute of Clinical Medicine of Chonbuk National University Hospital-Chonbuk National University, jeonju, South Korea, ²Division of Rheumatology, Department of Internal Medicine, Chonbuk National University Medical School and Research Institute of Clinical Medicine of Chonbuk National University Hospital-Chonbuk National University, JeonJu, South Korea, ³Internal Medicine, Presbyterian Medical center, Jeonju, South Korea, ⁴Department of Internal Medicine, School of Medicine, Wonkwang University, Iksan, Chonbuk, South Korea, ⁵Internal Medicine, School of Medicine, Wonkwang University, Iksan, Chonbuk, South Korea, ⁶Division of Rheumatology, Department of Internal Medicine, School of Medicine, The Catholic University of Korea, Seoul St. Mary's Hospital, Seoul, South Korea, ⁷Division of Rheumatology, Department of Internal Medicine, Gyeongsang National University School of Medicine, Jinju, South Korea, ⁸Department of Laboratory medicine, Chonbuk National University Medical School and Hospital, Jeonju, South Korea, ⁹Division of Rhuematology, Department of Internal Medicine, Chonbuk National University Medical School and Research Institute of Clinical Medicine of Chonbuk National University Hospital-Chonbuk National University, Jeonju, South Korea

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: Behcet's syndrome

Session Information

Date: Tuesday, November 10, 2015

Title: Vasculitis Poster III

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose: Behcet’s disease (BD) is a systemic vasculitis, whereas myelodysplastic syndrome (MDS) is a heterogeneous group of clonal hematologic disorders characterized by ineffective hematopoiesis. Some patients with BD comorbid with MDS involving trisomy 8 have intestinal lesions refractory to conventional medical therapies. We analyzed BD to evaluate the concurrence of BD and trisomy 8 and to determine whether trisomy 8 plays a role in the expression of BD associated with MDS.

Methods: Ninety-five consecutive patients were assessed for the presence of trisomy 8 in the peripheral blood. The clinical manifestation and genetic components were analyzed in patients with BD and MDS.

Results: Six of 95 patients were positive for Trisomy 8. Three of these cases were diagnosed with MDS, and they underwent bone marrow transplantation. The prevalence of MDS was as high as 50% in BD patients with trisomy 8. Gastrointestinal (GI) involvement was more common in patients with BD involving trisomy 8 than in non-trisomy8 patients (66% vs. 25%). The mean corpuscular volume (MCV) was higher in the presence of trisomy 8 than in cases lacking trisomy 8 (100.1 fl vs. 92.64 fl, P<0.01).

Conclusion: The prevalence of MDS was more higher in BD patients with trisomy 8. GI involvement seems to be an inherent feature of BD associated with MDS involving trisomy 8. When a patient with BD has increased MCV, the presence of trisomy 8 should be evaluated. Additionally, close monitoring for the possibility of MDS is necessary.

Disclosure: W. S. Lee, None; Y. J. Choi, None; M. J. Hong, None; C. H. Lee, None; M. S. Lee, None; Y. S. Suh, None; S. I. Lee, None; Y. G. Cho, None; W. H. Yoo, None.

To cite this abstract in AMA style:

Lee WS, Choi YJ, Hong MJ, Lee CH, Lee MS, Suh YS, Lee SI, Cho YG, Yoo WH. The Association Between Behcet’s Disease, Myelodysplastic Syndrome and Trisomy 8 [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/the-association-between-behcets-disease-myelodysplastic-syndrome-and-trisomy-8/. Accessed .

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