Clinical and Disease Characteristics From REVEAL at Time of Enrollment (Baseline): Prospective Observational Study of Patients With Polycythemia Vera in the United States

Clin Lymphoma Myeloma Leuk. 2018 Dec;18(12):788-795.e2. doi: 10.1016/j.clml.2018.08.009. Epub 2018 Aug 29.


Background: Polycythemia vera (PV) has a prevalence of 44 to 57 per 100,000 people in the United States. Prospective data concerning the demographics, clinical characteristics, and treatment patterns of patients with PV in the United States are lacking.

Patients and methods: The ongoing, prospective, observational REVEAL study evaluates demographics, disease burden, clinical management, patient-reported outcomes, and health care resource utilization of adult patients with PV in the United States. This report summarizes the demographics and clinical characteristics of patients at enrollment (baseline).

Results: Patients (n = 2510) were a median age of 67.0 years, 54.2% were male, and 89.1% were white. The median time from PV diagnosis to study enrollment was 4.0 (range, 0-56.3) years. Most patients (89.7%) were diagnosed after an abnormal blood test. Less than half (49.2%) underwent JAK2 mutation analysis, of whom 95.8% were JAK2 V617F mutation positive; < 1% were positive for JAK2 exon 12 mutations. At enrollment, 47.7% of patients had elevated hematocrit (> 45%), 35.8% had elevated platelets (> 400 × 109/L), and 37.0% had elevated leukocytes (> 10 × 109/L). Most patients (94.5%) were receiving active PV treatment, predominantly therapeutic phlebotomy alone (33.6%), hydroxyurea monotherapy (29.0%), or hydroxyurea plus phlebotomy (23.7%). Thrombotic events occurred in 11.9% of patients before PV diagnosis (venous, 6.7%; arterial, 5.7%), and 8.3% between diagnosis and enrollment. Hypertension (70.6%) was the most common previous medical condition.

Conclusion: REVEAL enrollment data inform our understanding of the baseline demographics, diagnostic approach, disease characteristics, and treatment patterns of patients with PV in the United States. Longitudinal real-world data collected in this study will complement information collected during randomized controlled clinical trials.

Keywords: Blood tests; Demographics; JAK2 V617F mutation; Myeloproliferative neoplasm; Population characteristics.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Biomarkers
  • Comorbidity
  • Female
  • Humans
  • Janus Kinase 2
  • Male
  • Middle Aged
  • Mutation
  • Myeloproliferative Disorders / diagnosis
  • Myeloproliferative Disorders / epidemiology
  • Myeloproliferative Disorders / etiology
  • Phenotype
  • Polycythemia Vera / diagnosis*
  • Polycythemia Vera / epidemiology*
  • Polycythemia Vera / etiology
  • Prospective Studies
  • Risk Factors
  • Socioeconomic Factors
  • Symptom Assessment
  • United States / epidemiology


  • Biomarkers
  • JAK2 protein, human
  • Janus Kinase 2