Essential Thrombocythemia Pathophysiology

ET Is Characterized by Bone Marrow and Blood Cell Abnormalities

Essential thrombocythemia (ET) is a rare but serious myeloproliferative neoplasm (MPN) characterized by thrombocytosis with bone marrow megakaryocytic hyperplasia and a tendency to develop thrombotic and hemorrhagic complications.1-3

Dysregulated JAK Signaling Plays a Role in the Pathophysiology of ET

As in myelofibrosis and polycythemia vera, dysregulated signaling in the JAK pathway plays a role in the pathophysiology of ET.4,5 The JAK2V617F mutation occurs in about 55% of patients with ET,6-8 and a mutation in the thrombopoietin receptor gene MPL occurs in about 4% of affected patients.9

Examining Bone Marrow
images of bone marrow comparing a normal marrow pattern to that of bone marrow with essential thrombocythemia disease
References

1. Cervantes F. Hematology. 2011;2011:215-221. 2. Tefferi A, Barbui T. Leukemia. 2013;27:1617-1620. 3. Wolanskyj AP, Schwager SM, McClure RF, et al. Mayo Clin Proc. 2006;81:159-166. 4. Quintás-Cardama A, Vaddi K, Liu P, et al. Blood. 2010;115:3109-3117. 5. Furqan M, Mukhi N, Lee B, Liu D. Biomark Res. 2013;1:5. 6. Vannucchi AM, Guglielmelli P, Tefferi A. CA Cancer J Clin. 2009;59:171-191. 7. Spivak JL, Silver RT. Blood. 2008;112:231-239. 8. Scott LM, Tong W, Levine RL, et al. N Engl J Med. 2007;1:356:459-468. 9. Beer PA, Green AR. Hematology Am Soc Hematol Educ Program. 2009:621-628. 10. Arber DA, Orazi A, Hasserjian R, et al. Blood. 2016;127(20):2391-2405.