[Refractory anemia complicated by Behçet's disease--report of three cases]

Three cases of refractory anemia (RA) are presented. They developed a complete, an intestinal, and an incomplete type of Behçet's disease after 13 years, 6 months, and 4 years of illness, respectively. They showed normal or only slightly reduced neutrophil counts in spite of anemia and thrombocytopenia. Although the precise etiology of Behçet's disease is still obscure, it is suggested that immunological reactions against Streptococcus viridans in chronic infection, and auto-oxidative damage induced by oxygen intermediates derived from stimulated neutrophils may play an important role in causing endothelial injury in the disease. Therefore, it is possible to speculate in our cases that the known susceptibility to bacterial infections in myelodysplastic syndromes, and the absence of neutropenia may have been responsible for the association of RA with Behçet's disease. It is also suggested that neutrophils are necessary for endothelial cell damage.