首页|Large-vessel thrombosis in intestinal Beh(c)et's disease complicated with myelodysplastic syndrome and trisomy 8

Large-vessel thrombosis in intestinal Beh(c)et's disease complicated with myelodysplastic syndrome and trisomy 8

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Beh(c)et's disease is characterized by recurrent oral ulcers,genital ulcers,uveitis and skin lesions.Myelodysplastic syndrome (MDS) is characterized by problems due to ineffective hematopoiesis.Several studies have identified a relationship between MDS and Beh(c)et's disease,especially intestinal Beh(c)et's disease.Trisomy 8 seems to play an important role in these disorders as well.The present case was a 24-year-old woman who had a huge tonsil ulcer with initial symptoms of odynophagia and intermittent fever.We also noted folliculitis on her upper back.Five days later,she began to experience diarrhea and abdominal pain.Abdominal computed tomography and subsequent surgery revealed ileum perforation and enterocolitis with multiple ulcers.Later,she was admitted again for a vulvar suppurative ulcer and suspicious Bartholin's cyst infection.The patient's clinical presentations met the criteria for Beh(c)et's disease.Six months after the bowel perforation event,we noted the development of pancytopenia in a routine laboratory examination.All the examinations led to the diagnosis of MDS with trisomy 8.The most unusual finding was that multiple large vessel thrombi developed during follow-up.Previous studies have suggested that trisomy 8 in MDS leads to concurrent intestinal Beh(c)et's disease.Moreover,the inflammatory and immune genes related to thrombus formation are overexpressed in cases of MDS with trisomy 8.Trisomy 8 must play a role in thrombosis.Further studies are needed to help clarify the pathophysiology and pathogenesis of these disorders.

Beh(c)et's diseaseMyelodysplastic syndromeTrisomy 8Intestinal ulcersThrombosis

Huang-Chi Chen、Ying-Ming Chiu

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Division of Critical Care Medicine, Department of Internal Medicine, Changhua Christian Hospital, 500Changhua, Taiwan, China

Division of Allergy, Immunology and Rheumatology, Department of Internal Medicine, Changhua Christian Hospital, 500 Changhua, Taiwan, China

2012

10.3748/wjg.v18.i10.1137

世界胃肠病学杂志(英文版)
太原消化病研治中心

世界胃肠病学杂志(英文版)

SCI
影响因子：1.001
ISSN：1007-9327
年,卷(期)：2012.18(10)
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