title: [Vasculitis associated with a myelodysplastic syndrome: a report of 5 cases].
abstract: In a study of 94 patients with myelodysplastic syndrome (MDS) associated vasculitis was observed in 5, which preceded hematologic diagnosis in 4. Leukocytoclastic vasculitis was observed in 5 cases being associated to lobular panniculitis in one. Three patients had refractory anemia, one sideroblastic anemia (SA) and another refractory anemia with excess blasts (RAEB). In the latter case lymphomatoid papulosis was also observed which, to date, has not been previously described in association with MDS. Another case presented seronegative polyarthritis and renal disease coinciding with vasculitis. Polyarteritis nodosa was diagnosed in a third patient in agreement with the criteria of the American College of Rheumatology, the association of which with MDS is exceptional. In the same case medullary cytogenetic study showed 46, XY,t (12;20), an abnormally which has not been described to date in cases of MDS. All the cases were treated with glucocorticoids in addition to cyclophosphamide in the patient with polyarteritis nodosa, with an improvement in the vasculitis being observed in all the patients. Two patients died, one (SA) due to pneumonia and the other (RAEB) due to subdural hematoma following transformation to acute myeloblastic leukemia. With 5% of the MDS studied presenting vasculitis, this syndrome should be included in the differential diagnosis of vasculitis observed in patients with cytopenia.
authors.affiliation: Servicio de Medicina Interna, Hospital Universitario 12 de Octubre, Madrid.
authors.forename: C
authors.initials: C
authors.lastname: Fernández-Miranda
authors.forename: A
authors.initials: A
authors.lastname: García-Marcilla
authors.forename: M
authors.initials: M
authors.lastname: Martín
authors.forename: R
authors.initials: R
authors.lastname: Gil
authors.forename: F
authors.initials: F
authors.lastname: Vanaclocha
authors.forename: N
authors.initials: N
authors.lastname: Torres
authors.forename: A
authors.initials: A
authors.lastname: del Palacio
mesh_terms: D000368:Aged; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D017731:Lymphomatoid Papulosis / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D010488:Polyarteritis Nodosa / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy
vernacular_title: Vasculitis asociada a un síndrome mielodisplásico: aportación de 5 casos.