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Clinico-Hematological Profile and Risk Stratification in Patients with Essential Thrombocythemia: Experience from Pakistan

  • Sultan, Sadia ;
  • Irfan, Syed Mohammed ;
  • Tanveer, Quratulain ;
  • Ali, Navaira
  • Published : 2015.12.03

Abstract

Background: Essential thrombocythemia (ET) is a Philadelphia chromosome-negative myeloproliferative neoplasm characterized by sustained thrombocytosis and megakaryocytic hyperplasia. It is an uncommon hematological malignancy which primarily affects elderly individuals. The rational of this study was to determine its clinico-hematological profile along with risk stratification in Pakistan patients. Materials and Methods: In this retrospective cross sectional study, 21 patients with ET were enrolled from January 2011 to December 2014. Data was analyzed with SPSS version 21. Results: The mean age was $56.7{\pm}19.0years$ (range 18-87) and the male to female ratio was 1:1.1. Of the total, 62% of patients were above 50 years of age. Overall 61.9% were diagnosed incidentally and were asymptomatic. In symptomatic patients, major complaints were weakness (19%); erythromelalgia (14.2%), transit ischemic attack (9.5%) and gastrointestinal bleed (4.7%). The mean hemoglobin count was $11.7{\pm}2.4g/dl$ with a total leukocyte count of $13.3{\pm}8.1{\times}10^9/l$ and platelets count of $1188.8{\pm}522.2{\times}10^9/l$. Serum lactate dehydrogenase, serum creatinine and uric acid were $454.3{\pm}127.8$, $1.2{\pm}0.5$ and $7.4{\pm}3.4$ respectively. According to risk stratification, 57.1% were in high risk; 23.8% in intermediate risk while 19.1% in low risk group. Conclusions: ET in our patients in Pakistan, unlike in the West, is seen in a relatively young population. Primarily patients were asymptomatic and risk stratification revealed predominance of high risk disease in our setting.

Keywords

Clinico-hematological;essential thrombocythemia;Pakistan

References

  1. Agarwal MB, Malhotra H, Chakrabarti P, et al (2015). Myeloproliferative neoplasms working group consensus recommendations for diagnosis and management of primary myelofibrosis, polycythemia vera, and essential thrombocythemia. Indian J Med Paediatr Oncol, 36, 3-16. https://doi.org/10.4103/0971-5851.151770
  2. Briere JB (2007). Essential thrombocythemia. Orphanet J Rare Dis, 2, 3. https://doi.org/10.1186/1750-1172-2-3
  3. Birgegard G (2015). Advances and challenges in the management of essential thrombocythemia. Ther Adv Hematol, 6, 142-56. https://doi.org/10.1177/2040620715580068
  4. Cervantes F (2011). Management of essential thrombocythemia. Hematol Am Soc Hematol Educ Program, 2011, 215-21. https://doi.org/10.1182/asheducation-2011.1.215
  5. Carneskog J, Wadenvik H, Fjalling M, Kutti J (1996). Assessment of spleen size using gamma camera scintigraphy in newly diagnosed patients with essential thrombocythaemia and polycythaemia vera. Eur J Haematol, 56, 158-62.
  6. Duangnapasatit B, Rattarittamrong E, Rattanathammethee T, et al (2015). Clinical manifestations and risk factors for complications of philadelphia chromosome-negative myeloproliferative neoplasms. Asian Pac J Cancer Prev, 16, 5013-8. https://doi.org/10.7314/APJCP.2015.16.12.5013
  7. Fabris F, Randi ML (2009). Essential thrombocythemia: past and present. Intern Emerg Med, 4, 381-8. https://doi.org/10.1007/s11739-009-0284-x
  8. Gangat N, Wolanskyj AP, McClure RF, et al (2007). Risk stratification for survival and leukemic transformation in essential thrombocythemia: a single institutional study of 605 patients. Leukemia, 21, 270-6. https://doi.org/10.1038/sj.leu.2404500
  9. Johansson P (2006). Epidemiology of the myeloproliferative disorders polycythemia vera and essential thrombocythemia. Semin Thromb Hemost, 32, 171-3. https://doi.org/10.1055/s-2006-939430
  10. Kiladjian JJ, Besses C, Griesshammer M, eta l (2013). Efficacy and safety of cytoreductive therapies in patients with essential thrombocythaemia aged >80 years: an interim analysis of the EXELS study. Clin Drug Investig, 33, 55-63. https://doi.org/10.1007/s40261-012-0042-0
  11. Lan HF, Fang ZH, Zhang Y, et al (2008). Clinical analysis of 438 patients with essential thrombocythemia. Zhonghua Xue Ye Xue Za Zhi, 29, 587-91.
  12. Ma X, Vanasse G, Cartmel B, Wang Y, Selinger HA (2008). Prevalence of polycythemia vera and essential thrombocythemia. Am J Hematol, 83, 359-62. https://doi.org/10.1002/ajh.21129
  13. Posfai E, Marton I, Nemes A, Borbenyi Z (2015). Thrombotic events and significance of the IPSET thrombosis risk evaluation score in essential thrombocythaemia. Orv Hetil, 156, 558-63. https://doi.org/10.1556/OH.2015.30117
  14. Sag SO, Gorukmez O, Ture M, et al (2015). MMP2 gene-735 C/T and MMP9 gene -1562 C/T polymorphisms in JAK2V617F positive myeloproliferative disorders. Asian Pac J Cancer Prev, 16, 443-9. https://doi.org/10.7314/APJCP.2015.16.2.443
  15. Sazawal S, Bajaj J, Chikkara S, et al (2010). Prevalence of JAK2 V617F mutation in Indian patients with chronic myeloproliferative disorders. Indian J Med Re,132, 423-7.
  16. Tefferi A, Barbui T (2015). Polycythemia vera and essential thrombocythemia: 2015 update on diagnosis, riskstratification and management. Am J Hematol, 90, 162-73. https://doi.org/10.1002/ajh.23895
  17. Thiele J, Kvasnicka HM, Orazi A, et al (2008). WHO classification of tumours of haemopoietic and lymphoid tissues. Lyon: International agency for research on cancer, 48.
  18. Varghese SJ, Bahey El Din M, Al Hendi M, Kumar R (2013). Essential thrombocythaemia: a single institution experience of 16 years. Indian J Hematol Blood Transfu, 29, 139-46. https://doi.org/10.1007/s12288-012-0172-9
  19. Yang JJ, Chen H, Zheng XQ, et al (2015). Methylated alteration of SHP1 complements mutation of JAK2 tyrosine kinase in patients with myeloproliferative neoplasm. Asian Pac J Cancer Prev, 16, 2219-25. https://doi.org/10.7314/APJCP.2015.16.6.2219
  20. Yonal-Hindilerden I, Daglar-Aday A, Akadam-Teker B, et al (2015). The burden of JAK2V617F mutated allele in turkish patients with myeloproliferative neoplasms. J Clin Med Res, 7, 161-70. https://doi.org/10.14740/jocmr2047w
  21. Zhang ZR, Duan YC (2014). Interferon apha 2b for treating patients with JAK2V617F positive polycythemia vera and essential thrombocytosis. Asian Pac J Cancer Prev, 15, 1681-4. https://doi.org/10.7314/APJCP.2014.15.4.1681