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Large-vessel thrombosis in intestinal Behçet's disease complicated with myelodysplastic syndrome and trisomy 8.

Abstract

Behçet'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 Behçet's disease, especially intestinal Behçet'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 Behçet'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 Behçet'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.

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  • Authors

    Chen HC, Chiu YM

    Source

    World journal of gastroenterology : WJG 18:10 2012 Mar 14 pg 1137-40

    MeSH

    Behcet Syndrome
    Chromosomes, Human, Pair 8
    Female
    Humans
    Myelodysplastic Syndromes
    Thrombosis
    Trisomy
    Young Adult

    Pub Type(s)

    Case Reports
    Journal Article

    Language

    eng

    PubMed ID

    22416191