Polycythemia Vera Management and Challenges in the Community Health Setting

Abstract

Patients with polycythemia vera (PV) experience shortened survival, increased risk of thromboembolic and hemorrhagic events, and burdensome symptoms. For all patients with PV, treatment with aspirin and hematocrit control with phlebotomy are recommended. In addition, patients with high-risk status or poor hematocrit control benefit from cytoreductive therapy with hydroxyurea, although approximately 1 in 4 patients develops resistance or intolerance. For patients who are resistant to or intolerant of hydroxyurea, studies have shown that ruxolitinib, a Janus kinase 1/2 inhibitor, provides hematocrit control, reduces spleen size, normalizes blood counts, and improves PV-related symptoms. For many patients, PV is managed in a community health setting, and it is important that community hematologists, oncologists, and internists are familiar with the contemporary management of PV to improve patient outcomes, including management for patients who present with unique health-care needs. This review provides an overview of current treatment options for patients with PV and discusses challenging circumstances encountered by community providers in the management of PV, including symptom assessment, identification of hydroxyurea resistance/intolerance, pregnancy, elective surgeries, concomitant immunosuppressants, and managing patients in areas with limited access to specialized hematologic care.

Evaluation of vascular events in patients with myeloproliferative syndromes and mutations of either the januskinase-2 or calreticulin gene at the university hospital Krems from 2008 to 2015

“…As mentioned before, high-risk patients are defined as older than 60 years and/or the presence of any prior arterial or venous vascular event [ 14 , 27 ]. The recommended treatment algorithm for patients of the high-risk category comprises low-dose ASA, phlebotomy (again in order to maintain hematocrit values below 45%) and Hydroxyurea [ 14 , 27 ]. Hydroxyurea further decreases the risk of vascular events by keeping the PLT count in a normal range, too [ 14 ].…”

Patterns of Hydroxyurea Prescription and Use in Routine Clinical Management of Polycythaemia Vera: a Multicentre Chart Review Study

“…Of the patients who were still on hydroxyurea therapy, 40.3% had a haematocrit concentration ≥45%, 29.2% had a platelet count >400×10 9 /L, and 34.4% had a WBC count >10x10 9 /L. Resistance/intolerance has been reported in nearly one fourth of the patients receiving hydroxyurea therapy (15,23). Hydroxyurea resistance/intolerance in PV patients is associated not only with an increased mortality rate but also with increased healthcare costs (24).…”

“…The classical risk factors for thrombotic events in PV patients include age of ≥60 years, history of thrombosis, and a hematocrit concentration of ≥45%. In addition, other risk factors still in need of validation have been reported, such as an elevated WBC count, female gender, mutant JAK2 allele burden, and classical cardiovascular risk factors [ 15 ]. In the present study, 50.9% of the patients were in the high-risk group (age ≥60 years and/or history of a thrombotic event).…”

“…Patients with PV are at higher risk of morbidity and mortality as compared with the general population. Thrombotic and hemorrhagic events are the major complications associated with mortality [ 15 ]. Treatment focuses on prevention of these complications.…”

“…Resistance/intolerance has been reported in nearly one-fourth of the patients receiving hydroxyurea therapy [ 15 , 23 ]. Hydroxyurea resistance/intolerance in PV patients is associated not only with an increased mortality rate but also with increased healthcare costs [ 24 ].…”