Keywords
risk factors
prognosis
How to Cite
Abstract
Objetive
Asses association between basal characteristics of patients with Essential Thrombocythemia (ET) and thromboembolism/bleeding/leukemic/fibrotic evolving.
Material and methods
An observational, retrospective study was conducted of prospectively captured data from patients with adult ET, followed up at the IHEMA Clinical Hematology Division between 01/01/1987 and 12/31/2017. The medical records of adult patients with ET who performed more than 2 post-diagnosis controls at the IIHEMA Hematology Clinic and with informed consent were reviewed. A review of the literature was also carried out in relation to the prognostic factors of this entity.
Results
The characteristics of 67 evaluable patients were: median (Md) 68-years-old, female 44, male 23, hematocrit Md 41%, hemoglobin Md 13.4 g/dL, leukocytes Md 9.7x109/L, platelets Md 852x109/L, splenomegaly 12, high risk 40, intermeddle 15, low 12, normal karyotype 40, JAK2V617Fpositive 29/41. Events: 67. Follow up Md 6 years. The most frequent event was bleeding (31%) and showed association to leukocytosis (Md 12.2x109/L in the group with bleeding and 8.8x109/L in the group without event (p=0.003)). Leukocytes >10.0x109/L was seen in 76% of the group with bleeding versus 36.96% of the group without event (p= 0.003). Thrombocytosis in the bleeding group was Md 1.204x109/L versus 814.5x109/L in the group without event (p=0.0098) but did not reach statistical significance when comparing to the proportion of patients with normal counts (p=0.46). Thromboembolism (24%) showed association to leukocytosis (Md 11.9x109/L in the group with thromboembolism and 9.2x109/L in the group without event (p=0.02). Leukocytosis >10.0x109/L was seen in 75% of the group with events and 41% of the group without event (p=0.018). Leukemia occurred in 4.4% and fibrosis 5.9%.
Conclusion
Events rate was similar to the literature. The only one basal characteristic associated to event was leukocytosis. JAK2 V617F mutation did not influence disease evolution.
References
2. Barbui T, Finazzi G, Carobbio A, et al. Development and validation of an International Prognostic Score of thrombosis in World Health Organization – essential thrombocythemia ( IPSET-thrombosis ). Blood. 2012;120(26):5128-5134.
3. Barbui T, Vannucchi AM, Buxhofer-Ausch V, et al. Practice-relevant revision of IPSET-thrombosis based on 1019 patients with WHO-defined essential thrombocythemia. Blood Cancer J. 2015;5(11):2-4.
4. Birgegard G. Advances and challenges in the management of essential thrombocythemia. Ther Adv Hematol. 2015;6(3):142-156.
5. Murphy S, Iland H, Rosenthal D, et al.Essential thrombocythemia: an interim report from the Polycythemia Vera Study Group.Semin Hematol.1986;23(3):177-82.
6. Jaffe ES, Harris NL, Stein H, et al. World Health Organization classification of tumours. Pathology and genetics of tumours of haematopoietic and lymphoid tissues 3rd ed. Lyon: (2001) IARC.
7. Swerdlow SH, Campo E, Harris NL et al. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. Lyon: (2008) IARC.
8. Swerdlow SH, Campo E, Harris NL et al. WHO classification of tumours of haematopoietic and lymphoid tissues. Revised 4th ed. Lyon: (2016) IARC.
9. Harrison CN, Bareford D, Butt N, et al. Guideline for investigation and management of adults and children presenting with a thrombocytosis. Br J Haematol. 2010;149(3):352-375.
10. Jones A, Kreil S, Zoi K. Widespread occurrence of the JAK2 V617F mutation in chronic myeloproliferative disorders. Blood. 2005;106(6):2162-2168.
11. Kim SY, Im K, Park SN, Kwon J, Kim JA, Lee DS. CALR, JAK2, and MPL mutation profiles in patients with four different subtypes of myeloproliferative neoplasms: Primary myelofibrosis, essential thrombocythemia, polycythemia vera, and myeloproliferative neoplasm, unclassifiable. Am J Clin Pathol. 2015;143(5):635-644.
12. Besses C, Cervantes F, Pereira A, et al. Major vascular complications in essential thrombocythemia: A study of the predictive factors in a series of 148 patients. Leukemia. 1999;13(2):150-154.
13. Barbui T, Barosi G, Birgegard G, et al. Philadelphia-negative classical myeloproliferative neoplasms: critical concepts and management recommendations from European leukemiaNet. J Clin Oncol. 2011;29(6):761-770.
14. Carobbio A, Thiele J, Passamonti F, et al. Risk factors for arterial and venous thrombosis in WHO-defined essential thrombocythemia: An international study of 891 patients. Blood. 2011;117(22):5857-5859.
15. Yang Y, Wang X, Wang C, Qin Y. A meta-analysis comparing clinical characteristics and outcomes in CALR-mutated and JAK2V617F essential thrombocythaemia. Int J Hematol. 2015;101(2):165-172.
16. Beer PA, Campbell PJ, Scott LM, et al. MPL mutations in myeloproliferative disorders: Analysis of the PT-1 cohort. Blood. 2008;112(1):141-149.
17. Palandri F, Polverelli N, Catani L, et al. Bleeding in essential thrombocythaemia: a retrospective analysis on 565 patients. Br J Haematol. 2012;156(2):279-281.
18. Finazzi G, Carobbio A, Thiele J, et al. Incidence and risk factors for bleeding in 1104 patients with essential thrombocythemia or prefibrotic myelofibrosis diagnosed according to the 2008 WHO criteria. Leukemia. 2012;26(4):716-719.
19. Michiels JJ. Erythromelalgic Thrombotic Thrombocythemia (ETT) and Hemorrhagic Trombocythemia (HT) in Patients with Essential Thrombocythaemia (ET) and Polycythaemia Vera (PV). J Hematol Thromboembolic Dis. 2014;2(6).
20. Alvarez-Larrán A, Pereira A, Guglielmelli P, et al. Antiplatelet therapy versus observation in low-risk essential thrombocythemia with a CALR mutation. Haematologica. 2016;101(8):926-931.
21. Barbui T, Thiele J, Passamonti F, et al. Survival and disease progression in essential thrombocythemia are significantly influenced by accurate morphologic diagnosis: A international study. J Clin Oncol. 2011;29(23):3179-3184.
22. Abdulkarim K, Ridell B, Johansson P, Kutti J, Safai-Kutti S, Andréasson B. The impact of peripheral blood values and bone marrow findings on prognosis for patients with essential thrombocythemia and polycythemia vera. Eur J Haematol. 2011;86(2):148-155.
23. Gangat N, Wolanskyj AP, McClure RF, et al. Risk stratification for survival and leukemic transformation in essential thrombocythemia: A single institutional study of 605 patients. Leukemia. 2007;21(2):270-276.
24. Tefferi A, Lasho TL, Guglielmelli P, et al. Targeted deep sequencing in polycythemia vera and essential thrombocythemia. Blood Adv. 2016;1(1):21-30.
25. Ruggeri M, Finazzi G, Tosetto A, Riva S, Rodeghiero F, Barbui T. No treatment for low-risk thrombocythaemia: Results from a prospective study. Br J Haematol. 1998;103(3):772-777.
26. Cortelazzo S, Finazzi G, Ruggeri M, et al. Hydroxyurea for patients with essential thrombocythemia and a high risk of thrombosis. N Engl J Med. 2001;332(17):1132-1136.
27. Harrison CN, Campbell PJ, Buck G, et al. Hydroxyurea Compared with Anagrelide in High-Risk Essential Thrombocythemia. N Engl J Med. 2005;353(1):33-45.
28. Campbell PJ, Maclean C, Beer PA, et al. Correlation of blood counts with vascular complications in essential thrombocythemia : analysis of the prospective PT1 cohort.Blood.2012;120(7):1409-1411.
All material published in the journal HEMATOLOGÍA (electronic and print version) is transferred to the Argentinean Society of Hematology. In accordance with the copyright Act (Act 11 723), a copyright transfer form will be sent to the authors of approved works, which has to be signed by all the authors before its publication. Authors should keep a copy of the original since the journal is not responsible for damages or losses of the material that was submitted. Authors should send an electronic version to the email: revista@sah.org.ar