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- Title
MPN-133: Mortality and Causes of Death of Patients with Polycythemia Vera: Analysis of the REVEAL Prospective, Observational Study.
- Authors
Stein, Brady L.; Patel, Kamal; Scherber, Robyn; Yu, Jingbo; Paranagama, Dilan; Miller, Carole B
- Abstract
Survival associated with polycythemia vera (PV) is often evaluated retrospectively, and cause-of-death is often poorly defined. To evaluate characteristics, survival-by-risk, and causes of death in patients with PV. An analysis of final data from the multicenter, non-interventional, Prospective Observational Study of Patients with Polycythemia Vera in US Clinical Practices (REVEAL; NCT02252159). US community and academic practices. Patients were followed during usual-care visits for 36 months until death, withdrawal, or study end. High-risk patients (per modified ELN criteria) were aged ≥60 years and/or had a thrombotic event (TE) history. Non-interventional. Demographic and clinical characteristics, survival, cause-of-death. Of 2510 patients enrolled between 7/2014 and 8/2016, 244 (9.7%) died in-study; 2266 (90.3%) were alive at last visit (mean follow-up, 110.3 and 179.4 weeks). Compared with living patients, those deceased were older at diagnosis (mean age, 68.5 vs 60.2 years; P<0.001), had longer disease duration (mean 6.5 vs 5.7 years, P=0.06); more were high-risk (82% vs 59.1%; P<0.001), and more had comorbidities (cardiac, 41.0% [100/244] vs 12.1% [275/2266]; blood/lymphatic [other than PV], 31.1% [76/244] vs 18.5% [419/2266]; vascular, 72.1% [176/244] vs 62.3% [1412/2266], neoplasms, 47.5% [116/244] vs 22.2% [503/2266]; respiratory, 55.7% [136/244] vs 33.4% [757/2266]; infections, 41.0% [100/244] vs 26.3% [597/2266]; all P<0.05). At death, 34.8% of patients experienced a TE; during the 6 months before death (n=190), 31.1%, 57.9%, and 36.8% had ≥1 elevated hematocrit, white blood cell (WBC) count, and platelet count, respectively (27.5% [52/189] had both ≥1 elevated WBC and platelet counts).The 4-year survival probability was 89% (95% CI, 0.87–0.90) and lower for high-risk vs low-risk patients (86% [0.85–0.88] vs 97% [0.95–0.98]; P<0.001). Most patients with known cause of death died from thrombotic complications (33.1% [58/175]). In this large contemporary analysis of patients with PV, estimated 4-year mortality was >10%, a surprising observation given the mean age of only ~66 years. Approximately one-third of patients died from thrombotic complications; >25% had elevated hematocrit or uncontrolled myeloproliferation in the 6 months before death. Deceased patients had higher-risk disease and higher comorbidity rates vs living patients. The observed high respiratory disorder rate in deceased patients is novel and warrants further investigation.
- Publication
Clinical Lymphoma, Myeloma & Leukemia, 2021, Vol 21, pS355
- ISSN
2152-2650
- Publication type
Article
- DOI
10.1016/S2152-2650(21)01820-6