Behçet's disease accompanied by myelodysplastic syndrome with trisomy 8: two case reports and a review of 15 Japanese cases

2003 ◽  
Vol 13 (1) ◽  
pp. 90-94 ◽  
Author(s):  
Y. Adachi ◽  
A. Tsutsumi ◽  
H. Murata ◽  
H. Takemura ◽  
Y. Chino ◽  
...  
Keyword(s):  
Myelodysplastic Syndrome ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Case Reports ◽  
Trisomy 8 ◽  
Behcet's Disease

scholarly journals A case of Behcet's disease associated with myelodysplastic syndrome involving trisomy 8 and a gain-of-function mutation in SHP-2

Rheumatology ◽  
2011 ◽  
Vol 50 (7) ◽  
pp. 1342-1344 ◽  
Author(s):  
T. Shigemura ◽  
K. Agematsu ◽  
T. Yamazaki ◽  
K. Sakashita ◽  
Y. Nakayama ◽  
...  
Keyword(s):  
Myelodysplastic Syndrome ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Trisomy 8 ◽  
Gain Of Function ◽  
Behcet's Disease

A Case of Behcet's Disease Associated with Schizophrenia and Myelodysplastic Syndrome with Trisomy 8 and 9

2013 ◽  
Vol 20 (6) ◽  
pp. 369
Author(s):  
Jung-Hoon Baek ◽  
Hyun-Jung Chun ◽  
Youn-Hee Park ◽  
Jeong-Hyeon Cho ◽  
Sang-Kyun Bae ◽  
...  
Keyword(s):  
Myelodysplastic Syndrome ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Trisomy 8 ◽  
Behcet's Disease

scholarly journals 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

2018 ◽  
Vol 2018 ◽  
pp. 1-8 ◽  
Author(s):  
Yan Shen ◽  
Hai-Fen Ma ◽  
Dan Luo ◽  
Jian-Fei Cai ◽  
Jun Zou ◽  
...  
Keyword(s):  
Myelodysplastic Syndrome ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Chinese Patients ◽  
Trisomy 8 ◽  
Ileocecal Region ◽  
Behcet's Disease ◽  
Cytogenetic Aberration ◽  
Cytogenetic Aberrations

This study aimed to investigate the characteristics of Chinese patients with Behçet disease (BD) and myelodysplastic syndrome (MDS) and explore the role played by trisomy 8. This was a retrospective study of patients with BD and MDS from the Shanghai Behçet’s disease database who were diagnosed between October 2012 and July 2017. There were 805 patients with BD and 16 also had MDS. Trisomy 8 was examined in patients with BD-MDS and some patients with gastrointestinal (GI) BD. Patients with BD and MDS (16/805; 2%) were more likely to be female and older; display fever and intestinal lesions; have lower leukocyte count, hemoglobin, platelet count; and show higher C-reactive protein and erythrocyte sedimentation rate (ESR) than patients with BD without MDS (all P<0.05). Trisomy 8 was common (81.3%) in patients with BD-MDS. Ulcers in the ileocecal region were more frequently seen in intestinal patients with BD-MDS than in BD without MDS (90.0% versus 48.9%; P=0.032). GI ulceration is common in patients with BD-MDS. Cytogenetic aberrations, especially trisomy 8, may play a role in the pathogenesis of intestinal involvement in patients with BD-MDS.

scholarly journals Intestinal Behçet’s disease complicated by myelodysplastic syndrome and secondary pulmonary alveolar proteinosis: a case report

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Hiroshi Shimizu ◽  
Shuzo Sato ◽  
Tomohiro Suzuki ◽  
Tomomi Sasajima ◽  
Yosuke Takahata ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Myelodysplastic Syndrome ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Chromosomal Abnormalities ◽  
Pulmonary Alveolar Proteinosis ◽  
Behçet's Disease ◽  
Trisomy 8 ◽  
Behcet's Disease ◽  
Alveolar Proteinosis

Abstract Background Gastrointestinal lesions, which sometimes develop in Behçet’s disease (BD), are referred to as intestinal BD. Although rare, intestinal BD can be accompanied by myelodysplastic syndrome (MDS) with abnormal karyotype trisomy 8, which is refractory to immunosuppressive therapy. Pulmonary alveolar proteinosis is a rare lung complication of BD and MDS. Herein, we present an extremely rare case of intestinal BD presenting with MDS and several chromosomal abnormalities, followed by secondary pulmonary proteinosis. Case presentation A 58-year-old Japanese woman with a 3-year history of genital ulcers and oral aphthae was admitted to our hospital. The patient developed abdominal pain and persistent diarrhea. Colonoscopy revealed multiple, round, punched-out ulcers from the terminal ileum to the descending colon. Intestinal BD was diagnosed and the patient was treated with colchicine, prednisolone, and adalimumab. However, her symptoms were unstable. Bone marrow examination to investigate the persistent macrocytic anemia revealed the presence of trisomy 8, trisomy 9, and X chromosome abnormalities (48, + 8, + 9, X, i(X) (q10) in 12 out of the examined 20 cells). Based on her hypoplastic bone marrow, the patient was diagnosed with low-risk MDS (refractory anemia). At the age of 61, the patient developed pneumonia with fever and diffuse ground-glass opacities on the lung computed tomography (CT). Chest high-resolution CT and histopathology via transbronchial lung biopsy revealed the presence of pulmonary alveolar proteinosis (PAP). These findings combined with the underlying disease led to the diagnosis of secondary PAP. Conclusions Secondary pulmonary proteinosis may accompany intestinal BD with MDS and several chromosomal abnormalities. Physicians should pay attention to lung complications, such as PAP, in patients with intestinal BD complicated by MDS. Genetic abnormalities may be associated with the development of such diseases.

scholarly journals Intestinal ulcers in a patient with myelodysplastic syndrome: a case report

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Xiaofen Zhang ◽  
Meng Jin ◽  
Zhe Shen ◽  
Xingyong Wan ◽  
Lan Li ◽  
...  
Keyword(s):  
Myelodysplastic Syndrome ◽  
Behçet’S Disease ◽  
Terminal Ileum ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Ileocecal Valve ◽  
Trisomy 8 ◽  
Behcet's Disease ◽  
Colon Capsule Endoscopy ◽  
Intestinal Ulcers

Abstract Background Trisomy 8 positivity myelodysplastic syndrome with Behçet's disease is rare. Isolated trisomy 8 is a frequent cytogenetic abnormality in the MDS, but the characteristic of trisomy 8 and the association between trisomy 8 positivity myelodysplastic syndrome and Behçet's disease is unclear. Case presentation Here, we reported a 63‐year‐old man, who presented with fever, abdominal pain and hematochezia. Imaging studies revealed bowel wall thickening and mural hyperenhancement of terminal ileum and cecum. Colonoscopy found multiple round ulcers in terminal ileum, ileocecal valve and multiple yellow dotted pseudomembranous attachments throughout the colon. Capsule endoscopy also revealed multiple irregular ulcers in lower ileum. Serum C-reactive protein levels and fecal calprotectin were abnormally high. The clostridium difficile toxin A and B was positive. However, the patient's intestinal ulcers did not resolve after two weeks course of vancomycin. Considered that the patient was diagnosed as MDS-RAEB2 with a karyotype of 47 XX, + 8. And detailed inquiry of medical history revealed epifolliculitis and frequently recurrent oral ulcers 2 months before admission. A diagnosis of trisomy 8 positivity MDS with BD was made. Then he received glucocorticoid along with the 5th course of azacytidine. The follow-up endoscopy showed significantly improved intestinal ulcer 2 months after treatment. we report a rare disease and provide the diagnose and treatment ideas. Conclusions We highlight the challenges and the process of thinking about of the diagnosis. This may provide a new idea for the diagnosis of intestinal ulcers.

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

2020 ◽  
Vol 18 (4) ◽  
pp. 469-475
Author(s):  
Yuki Mori ◽  
Fumihiko Iwamoto ◽  
Yasuaki Ishida ◽  
Toru Kuno ◽  
Shoji Kobayashi ◽  
...  
Keyword(s):  
Myelodysplastic Syndrome ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Bowel Perforation ◽  
Immunosuppressive Agents ◽  
Immunosuppressive Treatment ◽  
Trisomy 8 ◽  
Long Term Outcome ◽  
Behcet's Disease

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.

scholarly journals Coexistence of gastrointestinal Behçet’s disease, myelodysplastic syndrome and trisomy 8: A case report

2021 ◽  
Vol 13 (3) ◽  
pp. 134-136
Author(s):  
Erdal Bodakçi ◽  
Nazife Şule Yaşar Bilge ◽  
Neslihan Andıç ◽  
Timuçin Kaşifoğlu
Keyword(s):  
Case Report ◽  
Myelodysplastic Syndrome ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Trisomy 8 ◽  
Behcet's Disease
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