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

Ra Incalzi, Vincenzo Arena, Arnaldo Capelli, Giovanni Gambassi

Risultato della ricerca: Contributo in rivistaArticolo in rivista

6 Citazioni (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.
Lingua originaleEnglish
pagine (da-a)674-679
Numero di pagine6
RivistaJournal of Internal Medicine
Stato di pubblicazionePubblicato - 2004


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


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