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 OJEpi  Vol.10 No.4 , November 2020
Incidence in Ph-Negative Myeloproliferative Neoplasms in Armenia from 2005 to 2019
Abstract: Introduction: Enhancements of laboratory diagnostics and the emergence of new therapies had a significant impact on the incidence, prevalence and survival of patients with myeloproliferative neoplasms (MPN). Published epidemiology data are scarce, and multiple sources are needed to assess the disease burden. The aim of our work was to identify the patterns and trends of incidence, prevalence and survival of patients with MPN in the Republic of Armenia (RA) for the period 2005-2019. Methods: The data from Hematology Center blood diseases register, Oncological Center cancer register, as well as the data from death registration were the basis of our research. Demographic data were obtained from National Statistical Office of RA. Results: Analysis of the data obtained has shown that during the reporting period the average annual incidence of MPN was 1.84 per 100.000 inhabitants, including 2.1 for males and 1.64 for females. Analysis of incidence rates of MPN in relation to sex and age in the period under study revealed high rates in patients in groups 65 - 74 (8.3) and 55 - 64 (5.12 per 100 thousand years), respectively. According to the data obtained in the group of patients with MPN, the high annual average incidence rates are noted in primary myelofibrosis (PMF) (1.09 in 2018) and polycythemia vera (PV) (0.89 in 2016), the lowest for essential thrombocythemia (ET) (0.7 in 2016) per 100.000 population, respectively. In comparing our data to those obtained for 1966-1971 and 1998-2004 periods, one may detect a statistically significant increase in the total incidence of PMF and PV (p < 0.001). Conclusions: Analysis of the incidence rate in MPNs adjusted for age and gender shown the prevalence in group 65 - 74 (8.3) and in group 55 - 64 (5.13) per 100,000 inhabitants. The peak of incidence rate for both males and females was the age 65 - 74 and the male female incidence ratio in this age group was 11.3:6.2. The increasing incidence rate in MPNs in Armenia depends on the improvement of laboratory diagnosis. Thrombotic complications are observed in patients with an MPN in 45.3% of cases. In most cases, thrombosis is the first clinical symptom of an MPN, which determines the need for the introduction into clinical practice of molecular genetic testing methods among patients with thrombosis, an increase in blood levels, splenomegaly for the early diagnosis of clonal hematopoiesis and the use of a targeted drug.
Cite this paper: Lusine, S. , Anahit, T. , Maria, B. , Anahit, S. , Alla, S. and Samvel, D. (2020) Incidence in Ph-Negative Myeloproliferative Neoplasms in Armenia from 2005 to 2019. Open Journal of Epidemiology, 10, 355-368. doi: 10.4236/ojepi.2020.104029.
References

[1]   Brière, J.B. (2007) Essential Thrombocythemia. Orphanet Journal of Rare Diseases, 2, Article No. 3.
https://doi.org/10.1186/1750-1172-2-3

[2]   Hoffman, R., Prchal, J.T., Samuelson, S., Ciurea, S.O. and Rondelli, D. (2007) Philadelphia Chromosome-Negative Myeloproliferative Disorders: Biology and Treatment. Biology of Blood and Marrow Transplantation, 13, 64-72.
https://doi.org/10.1016/j.bbmt.2006.11.003

[3]   Scott, L.M., Beer, P.A., Bench, A.J., Erber, W.N. and Green, A.R. (2007) Prevalence of JAK2 V617F and Exon 12 Mutations in Polycythaemia Vera. British Journal of Haematology, 139, 511-512.
https://doi.org/10.1111/j.1365-2141.2007.06806.x

[4]   Vannucchi, A.M., Antonioli, E., Guglielmelli, P., et al. (2007) Prospective Identification of High-Risk Polycythemia Vera Patients Based on JAK2V617F Allele Burden. Leukemia, 21, 1952-1959.
https://doi.org/10.1038/sj.leu.2404854

[5]   Rollison, D.E., Howlader, N., Smith, M.T., Strom, S.S., Merritt, W.D., Ries, L.A., Edwards, B.K. and List, A.F. (2008) Epidemiology of Myelodysplastic Syndromes and Chronic Myeloproliferative Disorders in the United States, 2001-2004, Using Data from the NAACCR and SEER Programs. Blood, 112, 45-52.
https://doi.org/10.1182/blood-2008-01-134858

[6]   Shuvaev, V., Martynkevich, I., Abdulkadyrova, A., et al. (2014) Ph-Negative Chronic Myeloproliferative Neoplasms—Population Analysis, a Single Center 10-Years’ Experience. Blood, 124, 55-56.
https://doi.org/10.1182/blood.V124.21.5556.5556

[7]   Tefferi, A. and Barbui, T. (2019) Polycythemia Vera and Essential Thrombocythemia: 2019 Update on Diagnosis, Risk-Stratification and Management. American Journal of Hematology, 94, 133-143.
https://doi.org/10.1002/ajh.25303

[8]   James, C., Ugo, V., Le Couedic, J.P., et al. (2005) A Unique Clonal JAK2 Mutation Leading to Constitutive Signalling Causes Polycythaemia Vera. Nature, 434, 1144-1148.
https://doi.org/10.1038/nature03546

[9]   Lippert, E., Boissinot, M., Kralovics, R., et al. (2006) The JAK2-V617F Mutatio Is Frequently Present at Diagnosis in Patients with Essential Thrombocythemia and Polycythemia Vera. Blood, 108, 1865-1867.
https://doi.org/10.1182/blood-2006-01-013540

[10]   Campbell, P.J., Scott, L.M., Buck, G., et al. (2005) Definition of Subtypes of Essential Thrombocythemia and Relation to Polycythaemia Vera Based on JAK2 V617F Mutation Status: A Prospective Study. Lancet, N366, 1945-193.
https://doi.org/10.1016/S0140-6736(05)67785-9

[11]   Kralovics, R., Guan, Y. and Prchal, J.T. (2002) Acquired Uniparental Disomy of Chromosome 9p Is a Frequent Stem Cell Defect in Polycythemia Vera. Experimental Hematology, 30, 229-236
https://doi.org/10.1016/S0301-472X(01)00789-5

[12]   Huang, L.J., Constantinescu, S.N. and Lodish, H.F. (2001) The N-Terminal Domain of Janus Kinase 2 Is Required For Golgi Processing and Cell Surface Expression of Erythropoietin Receptor. Molecular Cell, 8, 1327-1338.
https://doi.org/10.1016/S1097-2765(01)00401-4

[13]   Ugo, V., Marzac, C., Teyssandier, I., Larbret, F., Lécluse, Y., Debili, N., Vainchenker, W. and Casadevall, N. (2004) Multiple Signaling Pathways Are Involved in Erythropoietin-Independent Differentiation of Erythroid Progenitors in Polycythemia Vera. Experimental Hematology, 32, 179-187.
https://doi.org/10.1016/j.exphem.2003.11.003

[14]   Mnjoyan, Z., Yoon, D., Li, J., et al. (2006) The Effect of the JAK2 V617F Mutation of PRV-1 Expression. Haematologica, 91, 411-412.

[15]   Baxter, E.J., Scott, L.M., Campbell, P.J., et al. (2005) Acquired Mutation of the Tyrosine Kinase JAK2 in Human Myeloproliferative Disorders. The Lancet, 365, 1054-1061.
https://doi.org/10.1016/S0140-6736(05)71142-9

[16]   Prchal, J. (2006) Does JAK2 1849G>T Initiate Polycythemia Vera? Blood, 108, 8-9.
https://doi.org/10.1182/blood-2006-04-016238

[17]   Kralovics, R., Passamonti, F., Buser, A.S., et al. (2005) A Gain-of-Function Mutation of JAK2 in Myeloproliferative Disorders. The New England Journal of Medicine, 352, 1779-1790.
https://doi.org/10.1056/NEJMoa051113

[18]   Popat, U., Frost, A., Liu, E., Guan, Y.L., Durette, A., Reddy, V. and Prchal, J.T. (2006) High Levels of Circulating CD34 Cells, Dacrocytes, Clonal Hematopoiesis, and JAK2 Mutation Differentiate Myelofibrosis with Myeloid Metaplasia from Secondary Myelofibrosis Associated with Pulmonary Hypertension. Blood, 107, 3486-3488.
https://doi.org/10.1182/blood-2005-08-3319

[19]   Onida, F., Ball, G., Kantarjian, H.M., et al. (2002) Characteristics and Outcome of Patients with Philadelphia Chromosome Negative, Bcr/Abl Negative Chronic Myelogenous Leukemia. Cancer, 95, 1673-1684.
https://doi.org/10.1002/cncr.10832

[20]   Tefferi, A. and Vardiman, J.M. (2008) Classification and Diagnosis of Myeloproliferative Neoplasms: The 2008 World Health Organization Criteria and Point-of-Care Diagnostic Algorithms. Leukemia, 22, 14-22.
https://doi.org/10.1038/sj.leu.2404955

[21]   Landgren, О., Goldin, L.R., Kristinsson, S.Y., Helgadottir, E.A., Samuelsson, J. and Björkholm, M. (2008) Increased Risks of Polycythemia Vera, Essential Thrombocythemia, and Myelofibrosis among 24577 First-Degree Relatives of 11039 Patients with Myeloproliferative Neoplasms in Sweden. Blood, 112, 2199-2204.
https://doi.org/10.1182/blood-2008-03-143602

[22]   Moulard, O., Mehta, J., Fryzek, J., Olivares, R., Iqbal, U. and Mesa, R.A. (2014) Epidemiology of Myelofibrosis, Essential Thrombocythemia, and Polycythemia Vera in the European Union. European Journal of Haematology, 92, 289-297.
https://doi.org/10.1111/ejh.12256

[23]   Vannucchi, A.M., Antonioli, E., Guglielmelli, P., Pardanani, A. and Tefferi, A. (2008) Clinical Correlates of JAK2V617F Presence or Allele Burden in Myeloproliferative Neoplasms: A Critical Reappraisal. Leukemia, 22, 1299-1307.
https://doi.org/10.1038/leu.2008.113

[24]   Hemminki, K., Zhang, H., Sundquist, J. and Bermejo, J.L. (2009) Myeloproliferative Disorders in Sweden: Incidence Trends and Multiple Tumors. Leukemia Research, 33, e14-e16.
https://doi.org/10.1016/j.leukres.2008.04.014

[25]   Lim, Y., Lee, J.-O. and Bang, S.-M. (2016) Incidence, Survival and Prevalence Statistics of Classical Myeloproliferative Neoplasm in Korea. Journal of Korean Medical Science, 31, 1579-1585.
https://doi.org/10.3346/jkms.2016.31.10.1579

[26]   Phekoo, K.J., Richards, M.A., Moller, H. and Schey, S.A. (2006) The Incidence and Outcome of Myeloid Malignancies in 2,112 Adult Patients in Southeast England. Haematologica, 91, 1400-1404.

[27]   Arber, D.A., Orazi, А. and Hasserjian, R. (2016) The 2016 Revision to the World Health Organization Classification of Myeloid Neoplasms and Acute Leukemia. Blood, 127, 2391-2405.
https://doi.org/10.1182/blood-2016-03-643544

[28]   Barosi, G., Bergamaschi, G., Marchetti, M., et al. (2007) JAK2 V617F Mutational Status Predicts Progression to Large Splenomegaly and Leukemic Transformation in Primary Myelofibrosis. Blood, 110, 4030-4036.
https://doi.org/10.1182/blood-2007-07-099184

[29]   Jensen, M.K., De Nully Brown, P., Nielsen, O.J. and Hasselbalch, H.C. (2000) Incidence, Clinical Features and Outcome of Essential Thrombocythaemia in a Well-Defined Geographical Area. European Journal of Haematology, 65, 132-139.
https://doi.org/10.1034/j.1600-0609.2000.90236.x

[30]   Vardiman, J.W., Thiele, J., Arber, D.A., et al. (2009) The 2008 Revision of the World Health Organization (Who) Classification of Myeloid Neoplasms and Acute Leukemia: Rationale and Important Changes. Blood, 114, 937-951.
https://doi.org/10.1182/blood-2009-03-209262

 
 
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