A case of Behçet's disease complicated with myelodysplastic syndrome

Behcet’s disease (BD) is a chronic systemic inflammatory vasculitis. Its pathogenesis includes neutrophil hyperfunction and the overproduction of inflammatory cytokines, including TNF-α. BD is often accompanied by leukocytosis, but is rarely associated with myelodysplastic syndrome (MDS). MDS is an acquired clonal hematopoietic stem cell disorder, characterized by ineffective, dysplastic hematopoiesis with peripheral cytopenia. The development of MDS is associated with genetic abnormalities, abnormal microenvironmental and immunological influences, and drugs. A few cases of BD complicated with MDS have been reported, many of which have intestinal ulcers, refractory anemia (RA), and a cytogenetic abnormality of trisomy 8. We present a case of BD complicated with MDS, which had intestinal ulcers unresponsive to immunosuppressive agents, and a subtype of RA with the double karyotypic abnormalities 1q duplication and Y deletion, instead of trisomy 8. (Korean J Med 75:S921-S925, 2008)