BEHCETS-DISEASE ASSOCIATED WITH MYELODYSPLASTIC SYNDROMES - A CASE-REPORT AND A REVIEW OF THE LITERATURE

BACKGROUND. Behcet's disease has rarely been reported in association w ith myelodysplastic syndromes (MDS). Increased production of reactive oxygen species (ROS) by neutrophils has a primary role in the pathogen esis of Behcet's disease. However, decreased production of ROS by neut rophils has frequently been reported in patients with MDS. The current study was undertaken to determine the role of ROS production in a pat ient with Behcet's disease and MDS. METHODS. A patient with MDS with t risomy 8 who developed Behcet's disease is described and a review of t he literature of patients with Behcet's disease in MDS is presented. T he production of ROS by neutrophils was investigated by luminol-enhanc ed chemiluminescence (CL) assay. RESULTS. Based on a review of the lit erature, 10 cases of Behcet's disease associated with MDS have been re ported to date. Nine patients had undergone cytogenetic analysis of bo ne marrow cells, 7 of whom (78%) had trisomy 8. Neutrophils taken from the authors' patient during the active phase of Behcet's disease demo nstrated an increased CL response. Moreover, serum from this patient i ncreased the CL emission of neutrophils from healthy volunteers. CONCL USIONS. These data suggest that trisomy 8 predisposes to Behcet's dise ase in patients with MDS. Furthermore, an increased ROS production by neutrophils may be associated with the diverse clinical findings in th is disease. In this study, neutrophils were activated directly by seru m factors. (C) 1997 American Cancer Society.