Paraneoplasic Behçet's syndrome associated with chronic myeloid leukemia

A 49-year-old man presented with a three-week history of abdominal pain, bloody diarrhoea and fever. Reported oral ulcers, weight loss and asthenia, as well as papulo-pustular lesions on his limbs and a recurrent ulcer in the lip (Fig.1) in the previous year. During hospitalization, he developed pathergy at venipuncture sites and painful scrotum ulcers. Laboratory showed pancytopenia and elevated CRP. Viral and autoimmune tests were negative. Abdominal CT revealed thickening of the ileocecal region with adenopathies. Blood smear and myelogram were compatible with Chronic Myeloid Leukemia (CML). Bone marrow culture and BK were negative. Karyotype revealed no changes, namely, no trisomy of the 8th. Ileocolonoscopy revealed aphthoid erosions of the ileocecal mucosa and ovoid punched-out cecal ulcers. Biopsies showed intense chronic inflammation in the lamina propria and submucosa with erosions and ulcers. Thus, presenting 5 points in the International Criteria for Behçet's Disease, this diagnosis was assumed as a paraneoplastic manifestation of CML. Corticosteroids improved symptoms, but the patient died three weeks later due to a blastic crisis. BS has been reported in association with CML, some concurrent with, or following treatment with interferon-a or hydroxyurea. Although the pathogenesis remains unclear, there is increasing awareness of its link to hematological malignancies, and trisomy of the 8th.