FRI0211 VASCULITIS ASSOCIATED WITH MYELODYSPLASTIC SYNDROME AND CHRONIC MYELOMONOCYTIC LEUKEMIA: FRENCH MULTICENTER CASE CONTROL STUDY

Background: Myelodysplastic syndromes (MDS) and MDS/myeloproliferative neoplasms (MDS/MPN) can be associated with vasculitis. Objectives: In this nationwide study by the “French Network of dysimmune disorders associated with hemopathies” (MINHEMON) the objective was to evaluate characteristics, treatment and outcome of vasculitis MDS-MDS/MPN. Methods: Retrospective analysis of patients that presented a MDS/MPN associated with vasculitis and compared the overall survival and acute leukemia with MDS without vasculitis. Results: Seventy patients with vasculitis and MDS/MPN were included, with a median age of 71.5 [21-90] years and male/female ratio of 2.3. Vasculitis was diagnosed prior to MDS/MPN in 31 patients (44.3%), with a median time of 27 months [1-120] between two diagnosis, and after in 20 patients (6 months [1-59]). In comparison to 183 MDS/MPN without dysimmune features showed no difference in MDS/MPN subtypes distribution nor median IPSS/CPSS scores in patients with and without vasculitis. The vasculitis subtypes was giant-cell arteritis (GCA) in 24 patients (34%). Eleven patients (20%) had Behcet’s-like syndrome and 6 patients (9%) presented with polyarteritis nodosa. Steroids (60 mg/day [0-500] of prednisone equivalent) were used as first-line therapy for MDS/MPN vasculitis in 64/70 patients (91%) and 41 (59%) received combined immunosuppressive therapies during the follow-up. After the follow-up of 33.2 months [1-162], 31 patients (44%) finally experienced sustained remission. At least one relapse during the 33.2 months [1-162] follow-up occurred in 43 patients (61%). Relapse rates were higher in patients treated by DMARDs (odds ratio at 4.86 [95% CI 1.38 - 17.10]), but did not differ from biologics (odds ratio 0.59 [95% CI 0.11-3.20]) and azacytidine (odds ratio 1.44 [95% CI 0.21-9.76]) (steroids considered as reference). Overall survival and progression to acute myeloid leukemia in MDS/MPN vasculitis were not significantly different from MDS/MPN patients without any dysimmune features (p=0.5). Conclusion: This first largest study of MDS/MPN vasculitis show no correlation of vasculitis subtypes with various subtypes and severity of MDS/MPN, and no significant impact of vasculitis on overall survival and progression to acute myeloid leukemia. The high relapse rats and steroid dependence raise the question of combined therapies to steroids. Whereas DMARDs use seem to be avoid specific azacytidine therapy could be considered for even low-risk MDS/MPN vasculitis. Acknowledgments: minhemon gfm gfev Disclosure of Interests: None declared