Gastrointestinal Behçet Disease Secondary to Myelodysplastic Syndrome

A 65-year-old man presented with low-grade fever, abdominal pain, and a 4-month history of extreme malaise. Complete blood count revealed severe anemia (hemoglobin 60 g/L; platelets 200 × 109/L; white blood cells 4.15 × 109/L), whereas a few blasts were noted on blood smear (Figure, A). Abdominal computed tomography revealed thickening of the ileocecal region (Figure, B). Endoscopy revealed multiple irregular ulcers with turbid appearance throughout the gastrointestinal (GI) mucosa (Figure, C), in addition to mucosal erosion and hemorrhage (Figure, D). Finally, bone marrow smear revealed 5% myeloblasts, whereas karyotyping demonstrated complex aberrations, including trisomy 8 (Figure, E). Myelodysplastic syndrome was diagnosed; accordingly, a hypomethylating agent significantly improved the patient's systemic symptoms and GI ulcerations.

F1FIGURE

Myelodysplastic syndrome is a myeloid neoplasm characterized by cytopenia and, when accompanied by trisomy 8, is often associated with autoimmune phenomena, especially GI Behçet disease. Patients with Behçet disease–like GI ulcerations and unexplained anemia should be screened for myelodysplastic syndrome.

Differential diagnoses: Crohn disease, ulcerative colitis, intestinal tuberculosis, intestinal malignancies.

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