LAUSR

A 76-year-old man presented to our hospital because of bloody stool and general fatigue. Colonoscopy revealed multiple erosions and edematous thickening in the ileocecal valve. Moreover, it also revealed multiple punched-out and oval-shaped ulcers in the ileocecal region (Fig. 1a,b,c,d). Thus, we suspected intestinal Behçet’s disease or simple ulcer. Histological findings indicated nonspecific inflammation (Fig. 2). On physical examination, the patient was afebrile and had no ocular symptoms, skin lesions, or genital ulcers. No active bleeding was noted; however, hemoglobin level was 6.0–7.0 g/dL. Furthermore, bone marrow aspiration was performed because the platelet level decreased from 131 000/μL to 77 000/μL and immature polymorphs (10.5%) were noted. Bone marrow aspiration revealed marrow dysplasia syndrome (MDS, RCMD) with monosomy 7 (Fig. 3). Based on these findings, he was diagnosed with intestinal Behçet’s disease —like ulcers associated with MDS with monosomy 7, and 5-aminosalicylic acid therapy was administered for ileocecal ulcers. Meanwhile, azacitidine was administered for MDS, which was ineffective as the patient died after 2 months. Several reports described a relationship between MDS, especially with trisomy 8, and intestinal Behçet’s disease. Because trisomy 8 induces abnormal inflammatory processes and immune gene expression, trisomy 8 in MDS could contribute to the aggravation of intestinal ulcers in patients with Behçet’s disease. In contrast, cases where intestinal Behçet’s disease was associated with MDS, including chromosome aberration, such as monosomy 7, except trisomy 8, were reported. Moreover, several cases of Behçet’s disease that presented with only ulcerative lesions of intestinal tract were reported without the main characteristic symptoms such as ocular lesion or skin lesion. Thus, when patients with MDS present digestive symptoms, such as bloody stools, regardless of the karyotype and typical symptoms of Behçet’s disease, intestinal Behçet’s disease associated with MDS should be considered.