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.
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