Clinical and Disease Characteristics of Patients With Polycythemia Vera in the United States
Polycythemia vera has a prevalence in the United States of 44 to 57 per 100,000 people.
A study published in Clinical Lymphoma, Myeloma, & Leukemia reported the demographics and clinical characteristics of patients with polycythemia vera (PV) at the time of enrollment into the Prospective Observational Study of Patients With Polycythemia Vera (REVEAL; ClinicalTrials.gov Identifier: NCT02252159), the first large, prospective observational study aimed at examining these characteristics in patients with PV in the United States.
The study enrolled 2510 patients with a clinical diagnosis of PV. Outcomes included demographics, PV disease characteristics, laboratory tests and procedures used to determine a PV diagnosis, signs and symptoms at time of diagnosis, comorbid conditions, and thrombotic event history.
The patients had a mean age of 66.3 years, 54.2% of the patients were male, and 89.1% of the patients were white. The median disease duration was 4.0 years, with family history of PV documented in 5.3% of patients. The most commonly reported PV-specific symptoms at diagnosis were tiredness, itching, muscle aches and bone pain, and difficulty sleeping. Janus kinase 2 (JAK2) mutational testing results were reported in 49.2% of patients, of whom 85.8% tested positive for JAK2 V617F and 0.8% tested positive for JAK2 exon 12 mutations.
At enrollment, the percentage of patients with low-risk PV (570 patients) being treated with therapeutic phlebotomy alone, hydroxyurea alone, phlebotomy plus hydroxyurea, and watchful waiting was 54.6%, 18.1%, 15.8%, and 4.9%, respectively. Of the 1940 patients with high-risk PV, 27.4%, 32.2%, and 5.7% were being managed with phlebotomy alone, hydroxyurea alone, and watchful waiting, respectively.
Mean hemoglobin, hematocrit, platelet, and leukocyte values at enrollment were 14.5 g/dL, 45%, 367.5 x 109/L, and 10.4 x 109/L, respectively. The most common comorbid conditions were hypertension, hyperlipidemia, obesity, and diabetes mellitus. At least 1 thrombotic event had been documented in 18.8% of patients for whom a PV diagnosis date was known; 279 patients experienced a venous thrombotic event, 218 patients experienced an arterial thrombotic event, and 29 patients experience both a venous and an arterial thrombotic event. Additionally, 667 patients reported at least 1 visit to a hospital, emergency room, urgent care clinic, or outpatient clinic in the 6 months prior to enrollment, and PV was a causal factor for 39.9% of the reported visits.
The authors noted that “demographic statistics from REVEAL are similar to those reported by other highly regarded studies,” but “the rates of JAK2 mutation testing reported in this study seem low” and “these data suggest variance from guideline-defined risk-adapted treatment of PV.” However, they expect that the information provided by REVEAL will improve clinicians' understanding of the demographics, diagnosis, management, and outcomes of patients with PV.
Disclosures: Multiple authors declare relationships with industry. Please refer to the original study for a full list of disclosures.
1. Grunwald MR, Stein BL, Boccia RV, et al. Clinical and disease characteristics from REVEAL at time of enrollment (Baseline): prospective observational study of patients with polycythemia vera in the United States [published online August 29, 2018]. Clin Lymphoma Myeloma Leuk. doi: 10.1016/j.clml.2018.08.009