Study of Patients with Polycythemia Vera in JAK2V617F and Mutated and Unmutated Status along with their Anxiety and Worried Thoughts: A Single-center Experience from Eastern India
Keywords :
Anxiety, JAK2V617F, Mutated, Unmutated, Polycythemia vera
Citation Information :
Vidhatri R, Saha S, Sharma A, Baul SN, Dolai TK. Study of Patients with Polycythemia Vera in JAK2V617F and Mutated and Unmutated Status along with their Anxiety and Worried Thoughts: A Single-center Experience from Eastern India. Bengal Physician Journal 2024; 11 (3):116-119.
Background: Polycythemia vera (PV) is associated with the gain of a function point mutation in Janus 2 tyrosine kinase (JAK-2), leading to an increase in the activity of JAK2V617F. The anxiety associated with PV is mostly incidentally detected post diagnosis.
Aim: To study the impact of JAK2V617F mutation status on the PV phenotype in terms of clinical and laboratory features. The mental anxiety of patients with PV was assessed at diagnosis and subsequently.
Material and methods: A retrospective study was done on 50 patients with PV for the last 5 years. For the measurement of anxiety among patients, a self-administered questionnaire was used as a screening tool and severity measure for generalized anxiety disorder questionnaire and scores were used and graded accordingly.
Results: On analysis of our 50 patients with PV. There was a significant statistical difference in terms of median age at presentation (61 vs 44.5 years; p = 0.002), median hemoglobin (Hb) at time of presentation (20.7 vs 17.5 gm/dL; p = 0.001), median total leukocyte count (TLC) at time of presentation (15,700 vs 8,100 µL; p = 0.001), median platelet counts at the time of presentation (3,20,000 vs 1,90,000 µL; p = 0.044) and low serum erythropoietin (EPO) level (p = 0.00) between JAK2 mutated and unmutated group. At one year when anxiety levels were checked in PV patients, it was found that none of the patients was severely anxious about their disease entity and few were moderately anxious.
Conclusion: The age of patients and laboratory parameters such as (Hb, TLC, platelet counts, and serum EPO levels) showed a statistically significant difference between JAK2 mutated and Unmutated group. Thrombosis was also more common in JAK2V617F mutated patients. With the passage of time gradually the stress and anxiety declined among patients with PV.
James C, Ugo V, Le Couédic JP, et al. A unique clonal JAK2 mutation leading to constitutive signalling causes polycythaemia vera. Nature 2005;434(7037):1144–1148. DOI: 10.1038/nature03546.
Kralovics R, Passamonti F, Buser AS, et al. A gain-of-function mutation of JAK2 in myeloproliferative disorders. N Engl J Med 2005;352(17):1779–1790. DOI: 10.1056/NEJMoa051113.
Levine RL, Wadleigh M, Cools J, et al. Activating mutation in the tyrosine kinase JAK2 in polycythemia vera, essential thrombocythemia, and myeloid metaplasia with myelofibrosis. Cancer Cell 2005;7(4):387–397. DOI: 10.1016/j.ccr.2005.03.023.
Baxter EJ, Scott LM, Campbell PJ, et al. Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders. Lancet 2005;365(9464):1054–1061. DOI: 10.1016/S0140-6736(05)71142-9.
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.
Spitzer RL, Kroenke K, Williams JB, et al. A brief measure for assessing generalized anxiety disorder: the GAD-7. Arch Intern Med 2006;166(10):1092–1097. DOI: 10.1001/archinte.166.10.1092.
Moliterno AR, Kaizer H, Reeves BN. JAK2 V617F allele burden in polycythemia vera: Burden of proof. Blood 2023;141(16):1934–1942. DOI: 10.1182/blood.2022017697.
Vannucchi AM, Pieri L, Guglielmelli P. JAK2 allele burden in the myeloproliferative neoplasms: Effects on phenotype, prognosis and change with treatment. Ther Adv Hematol 2011;2(1):21–32. DOI: 10.1177/2040620710394474.
Vannucchi AM. JAK2 mutation and thrombosis in the myeloproliferative neoplasms. Curr Hematol Malig Rep 2010;5:22–28. DOI: 10.1007/s11899-009-0038-x.
Passamonti F, Rumi E, Pietra D, et al. A prospective study of 338 patients with polycythemia vera: The impact of JAK2 (V617F) allele burden and leukocytosis on fibrotic or leukemic disease transformation and vascular complications. Leukemia 2010;24:1574–1579. DOI: 10.1038/leu.2010.148.
Gibek K, Sacha T, Cyranka K. Comparison of depressive, anxiety, and somatic symptoms in patients with essential thrombocythemia, polycythemia vera, and myelofibrosis treated with interferon alpha. Acta Haematol Polonica 2022;54:18–30. DOI: 10.5603/AHP.a2022.0058.
Landolfi R, Marchioli R, Kutti J, et al. Efficacy and safety of low-dose aspirin in polycythemia vera. N Engl J Med 2004;350:114–124. DOI: 10.1056/NEJMoa035572.
Barbui T, Carobbio A, Ghirardi A, et al. No correlation of intensity of phlebotomy regimen with risk of thrombosis in polycythemia vera: Evidence from European Collaboration on low-dose aspirin in polycythemia vera and cytoreductive therapy in polycythemia vera clinical trials. Haematologica 2017;102:e219–e221. DOI: 10.3324/haematol.2017.165126.
McMullin MF, Harrison CN, Ali S, et al. A guideline for the diagnosis and management of polycythaemia vera. A British Society for Haematology Guideline. Br J Haematol 2019;184:176–191. DOI: 10.1111/bjh.15648.
Arya Y, Syal A, Gupta M, et al. Advances in the treatment of polycythemia vera: Trends in disease management. Cureus 2021; 13(3):e14193. DOI: 10.7759/cureus.14193.