Isolated PACNS-like presentation of a systemic vasculitis complicating a myelodysplastic syndrome
| Autor: | Vincenzo Arena, Giovanni Gambassi, Raffaele Antonelli Incalzi, Arnaldo Capelli |
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| Rok vydání: | 2004 |
| Předmět: |
Pathology
medicine.medical_specialty Context (language use) Autopsy Fatal Outcome hemic and lymphatic diseases Internal Medicine medicine Humans Vasculitis Central Nervous System Aged business.industry Myelodysplastic syndromes Settore MED/09 - MEDICINA INTERNA medicine.disease Coronary Vessels medicine.anatomical_structure Myelodysplastic Syndromes Female Bone marrow Differential diagnosis Vasculitis Complication business Immunosuppressive Agents Systemic vasculitis |
| Zdroj: | Journal of Internal Medicine. 255:674-679 |
| ISSN: | 1365-2796 0954-6820 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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