Polycythemia Vera Post References

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  1. References:

  2. Barosi G et al. A unified definition of clinical resistance and intolerance to hydroxycarbamide in polycythaemia vera and primary myelofibrosis: results of a European LeukemiaNet (ELN) consensus process. British Journal of Haematology. 2010;148(6):961-963.

  3. Marchioli R et al. Cardiovascular events and intensity of treatment in polycythemia vera. The New England Journal of Medicine. 2013;368(1):22-33.

  4. Barbui T et al. White blood cell counts and thrombosis in polycythemia vera : a subanalysis of the CYTO-PV study. Blood. 2015;126(4):560-561.

  5. Emanuel RM 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. Journal of Clinical Oncology. 2012;30(33):4098-4103.

  6. Geyer H et al. Symptomatic profiles of patients with polycythemia vera: implications of inadequately controlled disease. Journal of Clinical Oncology. 2016;34(2):151-159.

  7. Parasuraman S et al. Patterns of hydroxyurea use and clinical outcomes among patients with polycythemia vera in real-world clinical practice: a chart review. Experimental Hematology & Oncology. 2016;5:3.

  8. Mascarenhas. A concise update on risk factors, therapy and outcome of leukemic transformation of myeloproliferative neoplasms. Clinical Lymphoma, Myeloma & Leukemia. 2016;16 suppl:S124-S129.

  9. Rumi E, Cazzola M. Diagnosis, risk stratification, and response evaluation in response evaluation in classical myeloproliferative neoplasms. Blood. 2017;129(6):680-692.

  10. Spivak JL et al. Two clinical phenotypes in polycythemia vera. The New England Journal of Medicine. 2014;371(9):808-817.


* In the Cytoreductive Therapy in Polycythemia Vera (CYTO-PV) study of 365 adult patients with PV treated with phlebotomy, hydroxyurea, or both, patients were randomized to 1 of 2 groups—either the low-Hct group (n = 182; with more intensive therapy to maintain a target Hct level <45%) or the high-Hct group (n = 183; with less intensive therapy to maintain a target Hct level of 45% to 50%). Baseline characteristics were balanced between the groups. Approximately 50% of patients had received an initial diagnosis of PV within 2 years prior to randomization. 67.1% of patients (n = 245) were at high risk because of age ≥65 years or previous thrombosis. The composite primary endpoint was the time until cardiovascular death or major thrombosis.²

† A prospective study of 1334 patients with PV was conducted to assess baseline symptoms with certain disease features (ie, known hydroxyurea use, known phlebotomy requirements, and splenomegaly). The patients had the following characteristics: known hydroxyurea use (n = 499), known phlebotomy (n = 646), palpable splenomegaly (n = 369), or all three features (n = 148). Assessment of myeloproliferative neoplasm (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).⁵

‡ Adjusted for age, gender, cardiovascular risk factors, previous thrombosis, and Hct levels.³