Pirayesh A, Verbunt R J, Kluin P M, Meinders A E, De Meijer P H
Department of General Internal Medicine, Leiden University Hospital, The Netherlands.
J Intern Med. 1997 Nov;242(5):425-31. doi: 10.1046/j.1365-2796.1997.00213.x.
Vasculitis in patients with the myelodysplastic syndrome (MDS) is a rare phenomenon. We describe a patient who presented with necrotic lesions of his toes, which proved to be the result of immune complex mediated vasculitis. This unusual combination of vasculitis and MDS prompted us to review the literature. Forty-four cases of vasculitis in association with MDS were found. The pathogenesis of the vasculitis in MDS remains speculative, although several reports suggest an immunological mechanism. The temporal relationship could not be determined in 20 (45%) of reported cases. The prognosis of these patients appears to be worse than in patients with MDS without vasculitis. Steroids were used in 41 (93%) of the reported cases. Our patient did not receive any drug therapy, nevertheless his necrotic lesions improved within a few weeks.
骨髓增生异常综合征（MDS）患者发生血管炎是一种罕见现象。我们描述了一名出现脚趾坏死性病变的患者，经证实这是免疫复合物介导的血管炎所致。血管炎与MDS这种不寻常的组合促使我们查阅文献。发现了44例与MDS相关的血管炎病例。尽管有几份报告提示存在免疫机制，但MDS中血管炎的发病机制仍具有推测性。在20例（45%）报告病例中无法确定时间关系。这些患者的预后似乎比无血管炎的MDS患者更差。在41例（93%）报告病例中使用了类固醇。我们的患者未接受任何药物治疗，但其坏死性病变在几周内有所改善。
