Association between Vasculitides and Myelodysplastic Syndrome (MDS): A Report on 21 Cases.

Vasculitis rarely complicates solid tumors but is more common in hematologic malignancies, particularly MDS,. We report characteristics and outcome of 21 patients with both MDS and vasculitis.

Patients: We retrospectively collected data of patients with at least 1 malignancy (including MDS) and vasculitis diagnosed between 1992 and 2002 in 6 French centers. Vasculitides occurring during cancer remission were excluded, as were giant-cell arteritis, relapsing polychondritis (a well known association with MDS), cryoglobulinemia, infection- or drug-induced vasculitis.

Results: 60 patients, with median follow-up of 45 months were analyzed. Vasculitides diagnosed were: cutaneous leukocytoclastic (LV,45%), polyarteritis nodosa (PAN,36.7%), Wegener’s granulomatosis (WG,6.7%), microscopic polyangiitis (MPA,5%), Henoch-Schonlein purpura( HSP,5%). Cancers were hematological (66.7%) (including 21 MDS (35%) and (23%) lymphoid malignancies) and solid tumors (41.7%). Five patients had 2 cancers.

21 patients had vasculitis associated with MDS (median age 66 yr; M/F ratio 4.25).

FAB classification was: RAEB (n=11), RA (n=3) and CMML (n=7). Vasculitides were LV (n=9), PAN (n=9), MPA(n=1), WG (n=1), cutaneous granulomatous vasculitis (n=1).CMML were only associated with necrotizing vasculitis ( 6PAN, 1WG). Vasculitis and MDS were diagnosed simultaneously in 12(57%) cases, vasculitis was diagnosed after MDS in 8 (38%) cases (median interval: 11 months, range 1–45) and 6 months before MDS in 1 case.9 MDS progressed to AML. Vasculitides developed before or at the time of leukemic progression in 6 of them (median interval 3 months, range 0–12). Main vasculitis symptoms were: fever (47%), skin lesions(81%), arthralgias (62%), peripheral neuropathy (14%), renal involvement (43%)[microaneurysms 29%, renal failure 9.5%, glomerulonephritis 9.5%], inflammatory syndrome (81%), ANCA (20%). Vasculitis therapy was steroids (90%) +/− immune suppressors (33%). MDS treatment was: AraC( n:2), VP16 (n:3), hydroxyurea (n:2),allogeneic BMT (n:2), symptomatic (n:12). Vasculitis was cured in 10 (47%) cases. 17 (81%) patients died; only 1 death was linked to vasculitis. Regardless of the type, vasculitides associated with MDS had significantly more frequent renal involvement (p=0.02),steroid dependence (p=0.04), and less recovery (p=0.04) than vasculitides without MDS

Conclusion: Vasculitides associated to MDS appear to have distinctive features and more severe evolution.In addition, occurrence of vasculitides during the course of MDS may be associated with a high risk of rapid leukemic progression.