Oostvogels R, Petersen E J, Chauffaille M L, Abrahams A C
Department of Haematology, University Medical Center Utrecht, the Netherlands.
Neth J Med. 2012 Mar;70(2):63-8.
The development of immunological abnormalities in various neoplasms is a rather common phenomenon. The prevalence of life-threatening systemic vasculitis in malignancy, however, is much lower. Nonetheless we found an unexpected frequency of several autoimmune manifestations, including systemic vasculitis, in certain myelodysplastic syndromes. We illustrate this finding with the case of a 43-year-old man with signs of polyarteritis nodosa-like systemic vasculitis during progression of chronic myelomonocytic leukaemia. Subsequently, we review the literature on the combination of myelodysplastic syndromes and systemic vasculitis and discuss the prognostic consequences, considerations for treatment and possible pathophysiological mechanisms.
各种肿瘤中免疫异常的发生是一种相当常见的现象。然而，恶性肿瘤中危及生命的系统性血管炎的发生率要低得多。尽管如此，我们发现在某些骨髓增生异常综合征中，包括系统性血管炎在内的几种自身免疫表现出现的频率出乎意料。我们以一名43岁男性为例来说明这一发现，该患者在慢性粒单核细胞白血病进展过程中出现了结节性多动脉炎样系统性血管炎的症状。随后，我们回顾了关于骨髓增生异常综合征与系统性血管炎合并存在的文献，并讨论了其预后后果、治疗考量及可能的病理生理机制。
