{"doi":"10.1002/ajh.26362","title":"Cause‐specific mortality following polycythemia vera, essential thrombocythemia, and primary myelofibrosis in the US population, 2001–2017","abstract":"Contemporary, population-based, cause-specific mortality risks within the heterogeneous subtypes of classical myeloproliferative neoplasms (MPNs)–polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF)–in the United States are needed to inform patient care. Therefore, we leveraged MPN cases diagnosed in the surveillance, epidemiology, and end results (SEER) Program in the 21st century to quantify cause-specific mortality risks and associated burden of death separately for PV, ET, and PMF, overall and by age and sex. We included all cases of first primary PV, ET, and PMF diagnosed during 2001–2016 (follow-up through 2017) among adult (ages 20–84 years) residents of 17 SEER cancer registry areas. Relative risk of death was estimated using standardized mortality ratios (SMRs; observed/expected), calculated by MPN subtype and stratified by age at diagnosis and sex. Differences in SMRs between subgroups were tested for significance using Poisson regression modeling and likelihood methods. We also calculated excess absolute risks (EAR) per 10 000 person-years and estimated MPN subtype-specific cumulative mortality. See the Methods section in Supporting Information for further details describing the SEER Program, study population, disease groupings, and statistical methods. We identified 13 340 individuals with PV, 12 346 individuals with ET, and 3230 individuals with PMF (Table 1). Mean follow-up time was 6.5, 6.0, and 4.0 years after a diagnosis of PV, ET, and PMF, respectively. MPNs accounted for < 10% of cause-specific death following a diagnosis of PV and ET and 34% following PMF (Table S1). All-cause mortality for each MPN exceeded that in the similarly aged general population, with 10-year cumulative mortality reaching 18.3%, 12.5%, and 48.5% for PV, ET, and PMF, respectively, for individuals < 60 years (general population: 6.8%) and 46.7%, 44.7%, and 83.7%, respectively, for those ≥ 60 years (general population: 29.1%) (Figure S1, Table S2). For patients with PV or ET, cumulative mortality for noncancer deaths far exceeded that for cancer deaths throughout 10 years of follow-up. In contrast, for PMF, cumulative incidence of deaths from all noncancers, all hematologic neoplasms (excluding MPN), and MPN were generally similarly elevated, whereas deaths from all solid tumors remained low throughout the follow-up period. Patients with PV had a 90% increased risk of death from all causes (excluding MPN), when compared to that expected in the general population (SMR = 1.9, 95% CI 1.8–2.0, EAR = 172.1; Table 1). Nearly 75% of these deaths (n = 2279; SMR = 2.0, EAR = 131.1) were due to noncancer causes, with 1.8–2.7-fold significantly increased SMRs observed for infections, including septicemia and respiratory infections; digestive diseases, including liver disease; cerebrovascular disease; cardiovascular diseases, including heart disease; and respiratory diseases, including chronic obstructive pulmonary disease (COPD); and a 4.3-fold elevated risk for benign hematologic disorders. The greatest burden of noncancer excess deaths/10 000 person-years was due to heart disease (EAR = 42.7), with lesser contributions from COPD, cerebrovascular disease, and infections (EARs = 11–21). Most of the relative and excess risks of deaths from neoplasms were accounted for by MDS/AML (SMR = 12.9, EAR = 17.6). In age and sex-specific analyses for PV, relative risks for all causes (excluding MPN) of death were higher among younger (SMR<60 = 2.8) compared with older individuals (SMR≥60 = 1.7, p < .001), but older patients experienced a disproportionate number of excess deaths (EAR<60 = 126.3 vs. EAR≥60 = 221.8; Table S3). Significantly higher SMRs due to cerebrovascular disease, heart disease, and COPD occurred among those diagnosed at ages < 60 versus ≥ 60 years, and significantly higher SMRs from heart disease, COPD, adverse events, and MDS/AML were observed among females than males. Heart disease had the highest EAR o","journal":"American Journal of Hematology","year":2021,"id":191290,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":13,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.8362,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2021-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":310851,"name":"Rochelle E. Curtis","orcid":"0000-0002-7883-5652","position":1,"is_corresponding":false},{"id":251241,"name":"Martha S. Linet","orcid":"0000-0002-1687-5587","position":2,"is_corresponding":false},{"id":108823,"name":"Lindsay M. Morton","orcid":"0000-0001-9767-2310","position":3,"is_corresponding":false},{"id":310850,"name":"Graça M. Dores","orcid":"0000-0002-3985-2935","position":0,"is_corresponding":true}],"reference_count":7,"raw_metadata":null,"created_at":"2026-07-18T23:49:31.121780Z","pmid":"34559904","pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}