Isolated PACNS-like presentation of a systemic vasculitis complicating a myelodysplastic syndrome

Ra Incalzi, Vincenzo Arena, Arnaldo Capelli, Giovanni Gambassi

Research output: Contribution to journalArticle

6 Citations (Scopus)


Myelodysplastic syndromes (MDS) are a series of haematological malignancies ranging from chronic refractory anaemia to leukaemia. There is increasing recognition of immunological abnormalities in patients with MDS, including few reports of cutaneous vasculitis; in no instance, a cerebral localization has been ascertained. Here, the case of a patient with MDS who presented exclusively with neurological signs that were considered indicative of a primary, isolated central nervous system vasculitis (PACNS) is reported. Although histological findings on brain tissue confirmed a small-vessel vasculitis, this had to be considered in the context of a systemic vasculitis. In fact, at autopsy, an involvement of skin, myocardium, lungs, liver, kidney and bone marrow was also found. An autoimmune vasculitis should be included in the differential diagnosis of acute-onset, isolated, cerebral symptoms complicating the course of MDS.
Original languageEnglish
Pages (from-to)674-679
Number of pages6
JournalJournal of Internal Medicine
Publication statusPublished - 2004


  • Aged
  • Coronary Vessels
  • Fatal Outcome
  • Female
  • Humans
  • Immunosuppressive Agents
  • Myelodysplastic Syndromes
  • Vasculitis, Central Nervous System


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