van Rijn R S, Wittebol S, Graafland A D, Kramer M H
Ziekenhuis Eemland, locatie De Lichtenberg, afd. Interne Geneeskunde, Amersfoort.
Ned Tijdschr Geneeskd. 2001 Aug 11;145(32):1529-33.
Two patients, men aged 77 and 66 years, presented with a vasculitis. Due to an abnormal blood smear they were referred to the internist, who subsequently diagnosed a myelodysplastic syndrome (MDS). They were initially treated with a high dose of corticosteroids and this dosage later became a maintenance dose. Immunological phenomena occur in 10-14% of MDS patients and seem to have a poor prognosis. Most patients respond well to treatment with corticosteroids. Occasionally, favourable haematological responses to corticosteroid therapy are seen, although this was not the case in the two patients described. When patients present with an immunological disorder, such as a vasculitis or an autoimmune disease, it is important to be aware of the possibility of an underlying MDS.
两名患者,分别为77岁和66岁男性,表现出血管炎症状。由于血液涂片异常,他们被转诊至内科医生处,随后被诊断为骨髓增生异常综合征(MDS)。他们最初接受了高剂量皮质类固醇治疗,该剂量后来成为维持剂量。免疫现象在10%至14%的MDS患者中出现,且预后似乎较差。大多数患者对皮质类固醇治疗反应良好。偶尔可见皮质类固醇治疗有良好的血液学反应,不过上述两名患者并非如此。当患者出现免疫紊乱,如血管炎或自身免疫性疾病时,必须意识到潜在MDS的可能性。