Year 2025 / Volume 117 / Number 7
Digestive Diseases Image
Paraneoplasic Behçet’s syndrome associated with chronic myeloid leukemia

399-400

DOI: 10.17235/reed.2023.10015/2023

Marta Moreira, Isabel Tarrio, Alda João Andrade, Luís Lopes,

Abstract
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.
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References
1 - Salihoglu, A., & Soysal, T. (2020). Trisomy-8-Positive Hematologic Malignancies Associated with Intestinal Behçet's Syndrome: Keep This Entity in Mind. Acta haematologica, 143(3), 194–195. https://doi.org/10.1159/000501841
2 - Cakmak, S. K., Gül, U., Kiliç, A., Gönül, M., Soylu, S., & Demirel, O. (2006). Behçet's disease associated with chronic myelogenous leukemia and chronic graft-vs-host disease. Leukemia & lymphoma, 47(12), 2674–2675. https://doi.org/10.1080/10428190600925426
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Moreira M, Tarrio I, Andrade A, Lopes L. Paraneoplasic Behçet’s syndrome associated with chronic myeloid leukemia. 10015/2023


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Publication history

Received: 11/10/2023

Accepted: 19/10/2023

Online First: 06/11/2023

Published: 08/07/2025

Article Online First time: 26 days

Article editing time: 636 days


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