Polycythemia Vera Symptom Burden

Polycythemia Vera (PV) Is Associated With Significant Symptom Burden

The signs and symptoms of PV may be similar to those of other myeloproliferative neoplasms.

Although patients with PV may be asymptomatic for many years, the disease is associated with substantial symptom burden.

Fatigue is the most common and most intense symptom reported.1

Symptoms can be associated with splenomegaly.2 Symptomatic or progressive splenomegaly may warrant further evaluation.


Self-Reported Symptoms of Polycythemia Vera1
image of graph that shows the percentage of self-reported polycythemia vera symptoms broken out by symptoms
Symptom assessment of 538 patients with polycythemia vera. Incidence is a score of >0 on the Myeloproliferative Neoplasm-Symptom Assessment Form Total Symptom Score (MPN-SAF TSS). Symptom severity was rated on a 0 (absent/as good as it can be) to 10 (worst imaginable/as bad as it can be) scale.1 The MPN-SAF TSS has a possible range of 0 to 100, with 100 representing the highest level of symptom severity. Fatigue was independently measured by coadministration of the Brief Fatigue Inventory.1

On Average, Patients With Known Hydroxyurea (HU) Use Had Moderately High Symptom Burden (Mean TSS = 29.2)3

  • A prospective study of 1334 patients with PV where a subset of patients received HU (n = 499)3*
Image of graph that shows MPN-10 mean symptom scores in patients with known HU use
Reprinted with permission. © 2016 American Society of Clinical Oncology. All rights reserved. MPN, myeloproliferative neoplasm; MPN-10, MPN Symptom Assessment Form; PBT, phlebotomy; TSS, Total Symptom Score. *A prospective study of 1334 patients with PV was conducted to assess baseline symptoms with certain disease features: known HU use (n = 499), known PBT (n = 646), palpable splenomegaly (n = 369), or all 3 features (n = 148), and compared to a control group of patients that lacked the specified feature. Assessment of MPN symptoms was performed by using the MPN-Symptom Assessment Form Total Symptom Score (MPN-SAF TSS; MPN-10). All items were evaluated on a 0 (absent) to 10 (worst imaginable) scale. The MPN-10 TSS has a possible range of 0 to 100 with 100 representing the highest level of symptom severity. The TSS for each patient was analyzed to place the patient into the quartiles of low symptom burden (TSS, 0 to 7), intermediate symptom burden (TSS, 8 to 17), moderately high symptom burden (TSS, 18 to 31), or high symptom burden (TSS ≥32).3


Patients With PV Had Moderately High Symptom Burden Regardless of Blood Count Control4

  • Symptom burden in patients who achieved blood count control versus those who did not was analyzed among 1813 evaluable patients with PV in the prospective, observational REVEAL study4†
Image of graph that shows Mean Total Symptom Score according to blood count control status (Hct, WBC, PLT)
Reprinted from Clinical Lymphoma Myeloma & Leukemia, 19(9), Grunwald MR, Burke JM, Kuter DJ, et al, Symptom burden and blood counts in patients with polycythemia vera in the United States: an analysis from the REVEAL Study, 579-584, Copyright 2019, with permission from Elsevier.
In the MPN Landmark Survey,
Image that shows – 66% of patients reported that their symptoms diminished their quality of life
CHR, complete hematologic remission; ET, essential thrombocythemia; Hct, hematocrit; MF, myelofibrosis; MPN, myeloproliferative neoplasm; MPN-SAF TSS, Myeloproliferative Neoplasm Symptom Assessment Form Total Symptom Score; PLT, platelet; WBC, white blood cell. REVEAL was a prospective, observational study that collected contemporary data regarding burden of disease, clinical management, patient-reported outcomes, and healthcare resource utilization from adult patients with PV in the United States, and was sponsored by Incyte. A total of 2510 patients were enrolled over an approximate 2-year period (July 2014 to August 2016), with 2307 patients having completed the MPN-SAF TSS at enrollment. Of these, 1813 (72.2%) had a complete blood count within 30 days before completion of the at-enrollment MPN-SAF TSS and were evaluable. At the time of enrollment, most patients (n = 1714; 94.5%) were being managed with cytoreductive therapy; 1581 patients (87.2%) were managed with phlebotomy, hydroxyurea, or a combination thereof. CHR was defined as Hct <45%, WBC count <10 × 109/L, and PLT count ≤400 × 109/L; these same criteria were used to determine if Hct, WBC, and PLT were controlled.4 The MPN Landmark Survey, funded by Incyte Corporation, was a web-based questionnaire composed of 65 multiple-choice questions intended to help evaluate the patient disease burden in the MPN disease setting. A total of 813 patients in the United States with a previous diagnosis of MF, PV, or ET completed the survey (MF, n = 207; PV, n = 380; ET, n = 226).6 §Patients reported whether they strongly agreed, somewhat agreed, somewhat disagreed, or strongly disagreed with the following statement: PV symptoms reduce my quality of life.6

1. Emanuel RM, Dueck AC, Geyer HL, et al. J Clin Oncol. 2012;30(33):4098-4103. 2. Barbui T, Barosi G, Birgegard G, et al. J Clin Oncol. 2011;29(6):761-770. 3. Geyer H, Scherber R, Kosiorek H, et al. J Clin Oncol. 2016;34(2):151-159. 4. Grunwald MR, Burke JM, Kuter DJ, et al. Clin Lymphoma Myeloma Leuk. 2019;19(9):579-584.e1. 5. Emanuel RM, Dueck AC, Geyer HL, et al. Blood. 2013;122(21):4067. 6. Mesa R, Miller CB, Thyne M, et al. BMC Cancer. 2016;16:167.