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Abstract
Behç et's disease is a multisystemic inflammatory disease characterized with recurrent oral ulcer, genital ulcer, and multiple organ involvement. Aplastic anemia is one of the rarest complications of Behç et's disease. There were only several reports about Behç et's disease associated myelodysplatic syndrome worldwide. Moreover, aplastic anemia in intestinal Behç et's disease was rarely reported. Here, we present a case of aplastic anemia with trisomy 8 and trisomy 9 in intestinal Behç et's disease and a review of the literatures. To the authors’ knowledge, this is the first case ever reported in Korea.
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Fig. 1.
Abdominal CT and colonoscopic findings. (A) Mildly enhanced and thickened wall involving terminal ileum suggests gastrointestinal involvement of Behç et's disease (arrows). (B) An excavating ulcer with discrete margin is noted in the terminal ileum (arrow).
Fig. 2.
Abdominal CT and surgical pathologic findings. (A) Right extended hemicolectomy status. The inflammatory wall thickening and ulceration of small bowel are aggravated, which is suggestive of disease progression. (B) The one of the surgical specimen is a segment of distal ileum, which is 11 cm in length and 4 cm in average diameter. A penetrating ulcer (3×2 cm) and three ulcerative lesions are noted.
Fig. 3.
Laboratory findings of complete blood counts. Despite of repetitive transfusion, leukopenia, anemia and thrombocytopenia were progressed. HOD, hospital day.
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