Oncology nurses have a key role in monitoring disease status of patients with polycythemia vera (PV), and can provide individualized approaches to help alleviate symptom burden for these patients, according to a review article published in the Clinical Journal of Oncology Nursing.1
Polycythemia vera is a Philadelphia chromosome-negative myeloproliferative neoplasm (MPN). Collectively, MPNs are rare bone marrow disorders characterized by the clonal proliferation of 1 or more types of hematopoietic stem cells. In particular, PV is characterized by an overproduction of erythrocytes, although high levels of white blood cells and platelets may also be present in patients with the condition.
According to the World Health Organization (WHO), the following 3 major criteria must be met before a diagnosis of PV can be made2:
- Hemoglobin level >16.5 g/dL in men, >16 g/dL in women; hematocrit >49% in men, >48% in women; or increased red blood cell mass.
- Tri-lineage proliferation with pleomorphic mature megakaryocytes in the bone marrow
- Disease characterized by a JAK2 mutation
“Because PV may progress slowly, nurses need to report abnormally elevated hemoglobin, hematocrit, and platelet levels, even if the patient appears asymptomatic, because complications may be avoided with early diagnosis and treatment,” noted Jill Brennan-Cook, DNP, who authored the review.
Complications of PV can include venous and arterial vascular events; hence, aspirin and cytoreductive agents, such as hydroxyurea and interferon, may be prescribed to decrease risk of a thromboembolic event in patients with this progressive disease.
Dr Brennan-Cook recommended following the National Comprehensive Cancer Network (NCCN) Clinical Practice Guidelines in Oncology for Myeloproliferative Neoplasms to facilitate standardized follow-up for patients with PV. The NCCN Guidelines outline approaches for monitoring, assessing, and managing bleeding, thrombosis, and other cardiovascular risk factors in these patients. Interventions may include smoking cessation and controlling hypertension and diabetes.3
Furthermore, PV is frequently associated with a high symptom burden, even in patients considered to have low-risk disease. These symptoms can adversely affect patient quality of life, as well as ability to perform activities of daily living and work-related responsibilities.
Common symptoms experienced by patients with PV include fatigue, night sweats, early satiety, pruritis, and difficulty concentrating. The MPN Symptom Assessment Form Total Symptom Score (MPN SAF TSS) is a validated tool designed for self-assessment of symptom severity and is also incorporated into the NCCN’s MPN guidelines.4 A review of patients’ results after completing the MPN SAF TSS can provide the basis for individualized patient education and interventions.
“Oncology nurses are well suited to assess for symptom burden and provide support with educational interventions to patients [with PV] and their families,” concluded Dr Brennan-Cook.1
1. Brennan-Cook J. Polycythemia vera: Symptom burden, oncology nurse considerations, and patient education. Clin J Oncol Nurs. 2020;24(5):575-578. doi:10.1188/20.CJON.575-578
2. Arber DA, Orazi A, Hasserjian R, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016;127(20):2391-2405. doi:10.1182/blood-2016-03-643544
3. National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Myeloproliferative Neoplasms. Accessed November 3, 2020. https://www.nccn.org/professionals/physician_gls/pdf/mpn.pdf
4. Emmanuel RM, Dueck AC, Geyer HL, et al. Myeloproliferative Neoplasm (MPN) Symptom Assessment Form Total Symptom Score: prospective international assessment of an abbreviated symptom burden scoring system among patients with MPNs. J Clin Oncol. 2012;30(33):4098-4103. doi:10.1200/JCO.2012.42.3863