Behcet'S-Like Syndrome And Other Dysimmunitary Manifestations Related To Myelodysplastic Syndromes With Trisomy 8

Myelodysplastic syndromes (MDS) are clonal hematopoietic malignancies which are also characterisedby immune dysregulation. The impaired immune response is mainly due to T lymphocytes (CD8 andT regulatory cells) with increased cell apoptosis. MDS could be associated in some cases with variousclinical dysimmune features; however, only MDS with trisomy 8 is correlated with particular clinicalphenotype. The latter is mainly Behcet's-like disease which includes orogenital aphtosis, skin featuresand severe ulcerative digestive disease of ileocaecal distribution. Other clinical manifestations, such asarthritis or neutrophilic dermatosis, have been also described in MDS patients with trisomy 8. The dysimmune manifestations, and among them the Behcet's-like disease, do not impact the overall survival or therisk of progression to acute myeloid leukemia. Immunosuppressive and immunomodulatory therapies, and among them TNF-alpha inhibitors, are usually ineffective to control the dysimmune manifestations. Targeting the underlying clonal disease with specific therapies, such as azacitidine, seems to be the beststrategy to control these disorders, even in MDS patients with low-risk disease. (C) 2020 Societe Nationale Francaise de Medecine Interne (SNFMI). Published by Elsevier Masson SAS. Allrights reserved.