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Published in: Journal of Thrombosis and Thrombolysis 4/2018

01-05-2018

Thrombosis in Philadelphia negative classical myeloproliferative neoplasms: a narrative review on epidemiology, risk assessment, and pathophysiologic mechanisms

Authors: Somedeb Ball, Kyaw Zin Thein, Abhishek Maiti, Kenneth Nugent

Published in: Journal of Thrombosis and Thrombolysis | Issue 4/2018

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Abstract

Thrombosis is common in cancer patients and is associated with increased morbidity and mortality. Myeloproliferative neoplasms (MPN) are common malignancies in elderly individuals and are known for a high incidence of thrombotic complications. Different risk factors have been identified in studies, and risk models have been developed to identify patients with MPN at higher risk for thrombosis. Several pathophysiological mechanisms help explain the increased likelihood of thrombosis in these patients. Factors, such as leukocyte and platelet activation leading to the formation of leukocyte–platelet aggregates, activation of the coagulation cascade by microparticles, high levels of inflammatory cytokines, and endothelial dysfunction have a crucial role in thrombosis in MPN patients. Recent studies have demonstrated a significant association between the allele burden of specific genetic mutations (mainly JAK2V617F) associated with MPN and the incidence of thrombotic events, thus suggesting a possible role for these mutations in thrombogenesis.
Literature
1.
go back to reference Arber DA, Orazi A, Hasserjian R et al (2016) The 2016 revision of the World Health Organization (WHO) classification of lymphoid neoplasms. Blood 127:2391–2405PubMedCrossRef Arber DA, Orazi A, Hasserjian R et al (2016) The 2016 revision of the World Health Organization (WHO) classification of lymphoid neoplasms. Blood 127:2391–2405PubMedCrossRef
2.
go back to reference Titmarsh GJ, Duncombe AS, McMullin MF et al (2014) How common are myeloproliferative neoplasms? A systematic review and metaanalysis. Am J Hematol 89:581–587PubMedCrossRef Titmarsh GJ, Duncombe AS, McMullin MF et al (2014) How common are myeloproliferative neoplasms? A systematic review and metaanalysis. Am J Hematol 89:581–587PubMedCrossRef
3.
go back to reference Moulard O, Mehta J, Fryzek J, Olivares R, Iqbal U, Mesa RA (2014) Epidemiology of myelofibrosis, essential thrombocythemia, and polycythemia vera in the European Union. Eur J Haematol 92:289–297PubMedCrossRef Moulard O, Mehta J, Fryzek J, Olivares R, Iqbal U, Mesa RA (2014) Epidemiology of myelofibrosis, essential thrombocythemia, and polycythemia vera in the European Union. Eur J Haematol 92:289–297PubMedCrossRef
4.
go back to reference Maynadié M, De Angelis R, Marcos-Gragera R et al (2013) Survival of European patients diagnosed with myeloid malignancies: a HAEMACARE study. Haematologica 98:230–238PubMedPubMedCentralCrossRef Maynadié M, De Angelis R, Marcos-Gragera R et al (2013) Survival of European patients diagnosed with myeloid malignancies: a HAEMACARE study. Haematologica 98:230–238PubMedPubMedCentralCrossRef
5.
go back to reference Hultcrantz M, Kristinsson SY, Andersson TM-L et al (2012) Patterns of survival among patients with myeloproliferative neoplasms diagnosed in Sweden from 1973 to 2008: a population-based study. J Clin Oncol 30:2995–3001PubMedPubMedCentralCrossRef Hultcrantz M, Kristinsson SY, Andersson TM-L et al (2012) Patterns of survival among patients with myeloproliferative neoplasms diagnosed in Sweden from 1973 to 2008: a population-based study. J Clin Oncol 30:2995–3001PubMedPubMedCentralCrossRef
6.
go back to reference Okamura T, Kinukawa N, Niho Y et al (2001) Primary chronic myelofibrosis: clinical and prognostic evaluation in 336 Japanese patients. Int J Hematol 73:194–198PubMedCrossRef Okamura T, Kinukawa N, Niho Y et al (2001) Primary chronic myelofibrosis: clinical and prognostic evaluation in 336 Japanese patients. Int J Hematol 73:194–198PubMedCrossRef
7.
go back to reference Antonioli E, Guglielmelli P, Pancrazzi A et al (2005) Clinical implications of the JAK2 V617F mutation in essential thrombocythemia. Leukemia 19:1847–1849PubMedCrossRef Antonioli E, Guglielmelli P, Pancrazzi A et al (2005) Clinical implications of the JAK2 V617F mutation in essential thrombocythemia. Leukemia 19:1847–1849PubMedCrossRef
8.
go back to reference Tefferi A (2010) Novel mutations and their functional and clinical relevance in myeloproliferative neoplasms: JAK2, MPL, TET2, ASXL1, CBL, IDH and IKZF1. Leukemia 24:1128–1138PubMedPubMedCentralCrossRef Tefferi A (2010) Novel mutations and their functional and clinical relevance in myeloproliferative neoplasms: JAK2, MPL, TET2, ASXL1, CBL, IDH and IKZF1. Leukemia 24:1128–1138PubMedPubMedCentralCrossRef
9.
go back to reference Barosi G, Bergamaschi G, Marchetti M et al (2007) Gruppo Italiano Malattie Ematologiche Maligne dell’Adulto (GIMEMA) Italian Registry of Myelofibrosis. JAK2 V617F mutational status predicts progression to large splenomegaly and leukemic transformation in primary myelofibrosis. Blood 110:4030–4036PubMedCrossRef Barosi G, Bergamaschi G, Marchetti M et al (2007) Gruppo Italiano Malattie Ematologiche Maligne dell’Adulto (GIMEMA) Italian Registry of Myelofibrosis. JAK2 V617F mutational status predicts progression to large splenomegaly and leukemic transformation in primary myelofibrosis. Blood 110:4030–4036PubMedCrossRef
10.
go back to reference Nangalia J, Massie CE, Baxter EJ et al (2013) Somatic CALR mutations in myeloproliferative neoplasms with nonmutated JAK2. N Engl J Med 369:2391–2405PubMedPubMedCentralCrossRef Nangalia J, Massie CE, Baxter EJ et al (2013) Somatic CALR mutations in myeloproliferative neoplasms with nonmutated JAK2. N Engl J Med 369:2391–2405PubMedPubMedCentralCrossRef
11.
go back to reference Tefferi A, Thiele J, Vannucchi AM, Barbui T (2014) An overview on CALR and CSF3R mutations and a proposal for revision of WHO diagnostic criteria for myeloproliferative neoplasms. Leukemia 28:1407–1413PubMedCrossRef Tefferi A, Thiele J, Vannucchi AM, Barbui T (2014) An overview on CALR and CSF3R mutations and a proposal for revision of WHO diagnostic criteria for myeloproliferative neoplasms. Leukemia 28:1407–1413PubMedCrossRef
12.
13.
14.
go back to reference Harrison C, Kiladjian JJ, Al-Ali HK (2012) JAK inhibition with ruxolitinib versus best available therapy for myelofibrosis. N Engl J Med 366:787–798PubMedCrossRef Harrison C, Kiladjian JJ, Al-Ali HK (2012) JAK inhibition with ruxolitinib versus best available therapy for myelofibrosis. N Engl J Med 366:787–798PubMedCrossRef
15.
go back to reference Tefferi A, Elliott M (2007) Thrombosis in myeloproliferative disorders: prevalence, prognostic factors, and the role of leukocytes and JAK2V617F. Semin Thromb Hemost 33:313–320PubMedCrossRef Tefferi A, Elliott M (2007) Thrombosis in myeloproliferative disorders: prevalence, prognostic factors, and the role of leukocytes and JAK2V617F. Semin Thromb Hemost 33:313–320PubMedCrossRef
16.
go back to reference Barbui T, Carobbio A, Cervantes F et al (2010) Thrombosis in primary myelofibrosis: incidence and risk factors. Blood 115:778–782PubMedCrossRef Barbui T, Carobbio A, Cervantes F et al (2010) Thrombosis in primary myelofibrosis: incidence and risk factors. Blood 115:778–782PubMedCrossRef
17.
go back to reference Gisslinger H, Gotic M, Holowiecki J et al; ANAHYDRET Study Group (2013) Anagrelide compared with hydroxyurea in WHO-classified essential thrombocythemia: the ANAHYDRET Study, a randomized controlled trial. Blood 121:1720–1728PubMedPubMedCentralCrossRef Gisslinger H, Gotic M, Holowiecki J et al; ANAHYDRET Study Group (2013) Anagrelide compared with hydroxyurea in WHO-classified essential thrombocythemia: the ANAHYDRET Study, a randomized controlled trial. Blood 121:1720–1728PubMedPubMedCentralCrossRef
18.
go back to reference Marchioli R, Finazzi G, Landolfi R et al (2005) Vascular and neoplastic risk in a large cohort of patients with polycythemia vera. J Clin Oncol 23:2224–2232PubMedCrossRef Marchioli R, Finazzi G, Landolfi R et al (2005) Vascular and neoplastic risk in a large cohort of patients with polycythemia vera. J Clin Oncol 23:2224–2232PubMedCrossRef
19.
go back to reference Buxhofer-Ausch V, Gisslinger H, Thiele J et al (2012) Leukocytosis as an important risk factor for arterial thrombosis in WHO-defined early/prefibrotic myelofibrosis: an international study of 264 patients. Am J Hematol 87:669–672PubMedCrossRef Buxhofer-Ausch V, Gisslinger H, Thiele J et al (2012) Leukocytosis as an important risk factor for arterial thrombosis in WHO-defined early/prefibrotic myelofibrosis: an international study of 264 patients. Am J Hematol 87:669–672PubMedCrossRef
20.
go back to reference Campbell PJ, Scott LM, Buck G et al (2005) Definition of subtypes of essential thrombocythaemia and relation to polycythaemia vera based on JAK2 V617F mutation status: a prospective study. Lancet 366:1945–1953PubMedCrossRef Campbell PJ, Scott LM, Buck G et al (2005) Definition of subtypes of essential thrombocythaemia and relation to polycythaemia vera based on JAK2 V617F mutation status: a prospective study. Lancet 366:1945–1953PubMedCrossRef
21.
go back to reference Montanaro M, Latagliata R, Cedrone M et al (2014) Thrombosis and survival in essential thrombocythemia: a regional study of 1,144 patients. Am J Hematol 89:542–546PubMedCrossRef Montanaro M, Latagliata R, Cedrone M et al (2014) Thrombosis and survival in essential thrombocythemia: a regional study of 1,144 patients. Am J Hematol 89:542–546PubMedCrossRef
22.
go back to reference De Stefano V, Martinelli I (2010) Splanchnic vein thrombosis: clinical presentation, risk factors and treatment. Intern Emerg Med 5:487–494PubMedCrossRef De Stefano V, Martinelli I (2010) Splanchnic vein thrombosis: clinical presentation, risk factors and treatment. Intern Emerg Med 5:487–494PubMedCrossRef
23.
go back to reference Barbui T, Thiele J, Carobbio A et al (2012) Disease characteristics and clinical outcome in young adults with essential thrombocythemia versus early/prefibrotic primary myelofibrosis. Blood 120:569–571PubMedCrossRef Barbui T, Thiele J, Carobbio A et al (2012) Disease characteristics and clinical outcome in young adults with essential thrombocythemia versus early/prefibrotic primary myelofibrosis. Blood 120:569–571PubMedCrossRef
24.
go back to reference Skeith L, Carrier M, Robinson SE, Alimam S, Rodger MA (2017) Risk of venous thromboembolism in pregnant women with essential thrombocythemia: a systematic review and meta-analysis. Blood 129:934–939PubMedCrossRef Skeith L, Carrier M, Robinson SE, Alimam S, Rodger MA (2017) Risk of venous thromboembolism in pregnant women with essential thrombocythemia: a systematic review and meta-analysis. Blood 129:934–939PubMedCrossRef
25.
go back to reference Khan I, Shergill A, Saraf SL et al (2016) Outcome disparities in Caucasian and non-Caucasian patients with myeloproliferative neoplasms. Clin Lymphoma Myeloma Leuk 16:350–357PubMedCrossRef Khan I, Shergill A, Saraf SL et al (2016) Outcome disparities in Caucasian and non-Caucasian patients with myeloproliferative neoplasms. Clin Lymphoma Myeloma Leuk 16:350–357PubMedCrossRef
26.
go back to reference Carobbio A, Thiele J, Passamonti F et al (2011) Risk factors for arterial and venous thrombosis in WHO-defined essential thrombocythemia: an international study of 891 patients. Blood 117:5857–5859PubMedCrossRef Carobbio A, Thiele J, Passamonti F et al (2011) Risk factors for arterial and venous thrombosis in WHO-defined essential thrombocythemia: an international study of 891 patients. Blood 117:5857–5859PubMedCrossRef
27.
go back to reference De Stefano V, Za T, Rossi E et al (2008) Recurrent thrombosis in patients with polycythemia vera and essential thrombocythemia: incidence, risk factors, and effect of treatments. Haematologica 93:372–380PubMedCrossRef De Stefano V, Za T, Rossi E et al (2008) Recurrent thrombosis in patients with polycythemia vera and essential thrombocythemia: incidence, risk factors, and effect of treatments. Haematologica 93:372–380PubMedCrossRef
28.
go back to reference De Stefano V, Rossi E, Za T et al (2011) JAK2 V617F mutational frequency in essential thrombocythemia associated with splanchnic or cerebral vein thrombosis. Am J Hematol 86:526–528PubMedCrossRef De Stefano V, Rossi E, Za T et al (2011) JAK2 V617F mutational frequency in essential thrombocythemia associated with splanchnic or cerebral vein thrombosis. Am J Hematol 86:526–528PubMedCrossRef
29.
go back to reference Ziakas PD (2008) Effect of JAK2 V617F on thrombotic risk in patients with essential thrombocythemia: measuring the uncertain. Haematologica 93:1412–1414PubMedCrossRef Ziakas PD (2008) Effect of JAK2 V617F on thrombotic risk in patients with essential thrombocythemia: measuring the uncertain. Haematologica 93:1412–1414PubMedCrossRef
30.
go back to reference Dahabreh IJ, Zoi K, Giannouli S, Zoi C, Loukopoulos D, Voulgarelis M (2008) Is JAK2 V617F mutation more than a diagnostic index? A meta-analysis of clinical outcomes in essential thrombocythemia. Leuk Res 33:67–73PubMedCrossRef Dahabreh IJ, Zoi K, Giannouli S, Zoi C, Loukopoulos D, Voulgarelis M (2008) Is JAK2 V617F mutation more than a diagnostic index? A meta-analysis of clinical outcomes in essential thrombocythemia. Leuk Res 33:67–73PubMedCrossRef
31.
go back to reference Lussana F, Caberlon S, Pagani C, Kamphuisen PW, Buller HR, Cattaneo M (2009) Association of V617F Jak2 mutation with the risk of thrombosis among patients with essential thrombocythaemia or idiopathic myelofibrosis: a systematic review. Thromb Res 124:409–417PubMedCrossRef Lussana F, Caberlon S, Pagani C, Kamphuisen PW, Buller HR, Cattaneo M (2009) Association of V617F Jak2 mutation with the risk of thrombosis among patients with essential thrombocythaemia or idiopathic myelofibrosis: a systematic review. Thromb Res 124:409–417PubMedCrossRef
32.
go back to reference Carobbio A, Finazzi G, Antonioli E et al (2009) JAK2V617F allele burden and thrombosis: a direct comparison in essential thrombocythemia and polycythemia vera. Exp Hematol 37:1016–1021PubMedPubMedCentralCrossRef Carobbio A, Finazzi G, Antonioli E et al (2009) JAK2V617F allele burden and thrombosis: a direct comparison in essential thrombocythemia and polycythemia vera. Exp Hematol 37:1016–1021PubMedPubMedCentralCrossRef
33.
go back to reference De Stefano V, Za T, Rossi E et al (2010) Leukocytosis is a risk factor for recurrent arterial thrombosis in young patients with polycythemia vera and essential thrombocythemia. Am J Hematol 85:97–100PubMed De Stefano V, Za T, Rossi E et al (2010) Leukocytosis is a risk factor for recurrent arterial thrombosis in young patients with polycythemia vera and essential thrombocythemia. Am J Hematol 85:97–100PubMed
34.
go back to reference Landolfi R, Di Gennaro L, Barbui T et al (2007) European collaboration on low-dose aspirin in polycythemia vera (ECLAP). Leukocytosis as a major thrombotic risk factor in patients with polycythemia vera. Blood 109:2446–2452PubMedCrossRef Landolfi R, Di Gennaro L, Barbui T et al (2007) European collaboration on low-dose aspirin in polycythemia vera (ECLAP). Leukocytosis as a major thrombotic risk factor in patients with polycythemia vera. Blood 109:2446–2452PubMedCrossRef
35.
go back to reference Carobbio A, Antonioli E, Guglielmelli P et al (2008) Leukocytosis and risk stratification assessment in essential thrombocythemia. J Clin Oncol 26:2732–2736PubMedCrossRef Carobbio A, Antonioli E, Guglielmelli P et al (2008) Leukocytosis and risk stratification assessment in essential thrombocythemia. J Clin Oncol 26:2732–2736PubMedCrossRef
36.
go back to reference Barbui T, Finazzi G, Carobbio A et al (2012) Development and validation of an International Prognostic Score of thrombosis in World Health Organization-essential thrombocythemia (IPSET thrombosis). Blood 120:5128–5133PubMedCrossRef Barbui T, Finazzi G, Carobbio A et al (2012) Development and validation of an International Prognostic Score of thrombosis in World Health Organization-essential thrombocythemia (IPSET thrombosis). Blood 120:5128–5133PubMedCrossRef
37.
go back to reference Alvarez-Larrán A, Pereira A, Arellano-Rodrigo E, Hernández-Boluda JC, Cervantes F, Besses C (2013) Cytoreduction plus low-dose aspirin versus cytoreduction alone as primary prophylaxis of thrombosis in patients with high-risk essential thrombocythaemia: an observational study. Br J Haematol 161:865–871PubMedCrossRef Alvarez-Larrán A, Pereira A, Arellano-Rodrigo E, Hernández-Boluda JC, Cervantes F, Besses C (2013) Cytoreduction plus low-dose aspirin versus cytoreduction alone as primary prophylaxis of thrombosis in patients with high-risk essential thrombocythaemia: an observational study. Br J Haematol 161:865–871PubMedCrossRef
38.
go back to reference Hernández-Boluda JC, Arellano-Rodrigo E, Cervantes F et al (2015) Oral anticoagulation to prevent thrombosis recurrence in polycythemia vera and essential thrombocythemia. Ann Hematol 94:911–918PubMedCrossRef Hernández-Boluda JC, Arellano-Rodrigo E, Cervantes F et al (2015) Oral anticoagulation to prevent thrombosis recurrence in polycythemia vera and essential thrombocythemia. Ann Hematol 94:911–918PubMedCrossRef
39.
go back to reference Dombi P, Illés Á, Demeter J et al (2017) Anagrelide reduces thrombotic risk in essential thrombocythaemia vs. hydroxyurea plus aspirin. Eur J Haematol 98:106–111PubMedCrossRef Dombi P, Illés Á, Demeter J et al (2017) Anagrelide reduces thrombotic risk in essential thrombocythaemia vs. hydroxyurea plus aspirin. Eur J Haematol 98:106–111PubMedCrossRef
40.
go back to reference Samuelson BT, Vesely SK, Chai-Adisaksopha C, Scott BL, Crowther M, Garcia D (2016) The impact of ruxolitinib on thrombosis in patients with polycythemia vera and myelofibrosis: a meta-analysis. Blood Coagul Fibrinolysis 27:648–652PubMedCrossRef Samuelson BT, Vesely SK, Chai-Adisaksopha C, Scott BL, Crowther M, Garcia D (2016) The impact of ruxolitinib on thrombosis in patients with polycythemia vera and myelofibrosis: a meta-analysis. Blood Coagul Fibrinolysis 27:648–652PubMedCrossRef
41.
go back to reference Afshar-Kharghan V, Thiagarajan P (2006) Leukocyte adhesion and thrombosis. Curr Opin Hematol 13:34–39PubMedCrossRef Afshar-Kharghan V, Thiagarajan P (2006) Leukocyte adhesion and thrombosis. Curr Opin Hematol 13:34–39PubMedCrossRef
42.
go back to reference Yakubenko VP, Lishko VK, Lam SC-T, Ugarova TP (2002) A molecular basis for integrin aMb2 ligand binding promiscuity. J Biol Chem 50:48635–48642CrossRef Yakubenko VP, Lishko VK, Lam SC-T, Ugarova TP (2002) A molecular basis for integrin aMb2 ligand binding promiscuity. J Biol Chem 50:48635–48642CrossRef
43.
go back to reference Vannucchi AM, Antonioli E, Guglielmelli P et al (2007) Prospective identification of high-risk polycythemia vera patients based on JAK2(V617F) allele burden. Leukemia 21:1952–1959PubMedCrossRef Vannucchi AM, Antonioli E, Guglielmelli P et al (2007) Prospective identification of high-risk polycythemia vera patients based on JAK2(V617F) allele burden. Leukemia 21:1952–1959PubMedCrossRef
44.
go back to reference Passamonti F, Rumi E, Pietra D et al (2006) Relation between JAK2(V617F) mutation status, granulocyte activation, and constitutive mobilization of CD34+ cells into peripheral blood in myeloproliferative disorders. Blood 107:3676–3682PubMedCrossRef Passamonti F, Rumi E, Pietra D et al (2006) Relation between JAK2(V617F) mutation status, granulocyte activation, and constitutive mobilization of CD34+ cells into peripheral blood in myeloproliferative disorders. Blood 107:3676–3682PubMedCrossRef
45.
go back to reference Cerletti C, Tamburrelli C, Izzi B, Gianfagna F, de Gaetano G (2012) Platelet–leukocyte interactions in thrombosis. Thromb Res 129:263–266PubMedCrossRef Cerletti C, Tamburrelli C, Izzi B, Gianfagna F, de Gaetano G (2012) Platelet–leukocyte interactions in thrombosis. Thromb Res 129:263–266PubMedCrossRef
46.
go back to reference Falanga A, Marchetti M, Evangelista V et al (2000) Polymorphonuclear leukocyte activation and hemostasis in patients with essential thrombocythemia and polycythemia vera. Blood 96:4261–4266PubMed Falanga A, Marchetti M, Evangelista V et al (2000) Polymorphonuclear leukocyte activation and hemostasis in patients with essential thrombocythemia and polycythemia vera. Blood 96:4261–4266PubMed
47.
go back to reference Brinkmann V, Reichard U, Goosmann C et al (2004) Neutrophil extracellular traps kill bacteria. Science 303:1532–1535PubMedCrossRef Brinkmann V, Reichard U, Goosmann C et al (2004) Neutrophil extracellular traps kill bacteria. Science 303:1532–1535PubMedCrossRef
49.
50.
go back to reference Demers M, Krause DS, Schatzberg D et al (2012) Cancers predispose neutrophils to release extracellular DNA traps that contribute to cancer-associated thrombosis. Proc Natl Acad Sci USA 109:13076–13081PubMedPubMedCentralCrossRef Demers M, Krause DS, Schatzberg D et al (2012) Cancers predispose neutrophils to release extracellular DNA traps that contribute to cancer-associated thrombosis. Proc Natl Acad Sci USA 109:13076–13081PubMedPubMedCentralCrossRef
51.
go back to reference Di Nisio M, Barbui T, Di Gennaro L et al (2007) European Collaboration on Low-dose Aspirin in Polycythemia Vera (ECLAP) Investigators. The haematocrit and platelet target in polycythemia vera. Br J Haematol 136:249–259PubMedCrossRef Di Nisio M, Barbui T, Di Gennaro L et al (2007) European Collaboration on Low-dose Aspirin in Polycythemia Vera (ECLAP) Investigators. The haematocrit and platelet target in polycythemia vera. Br J Haematol 136:249–259PubMedCrossRef
52.
go back to reference Marchioli R, Finazzi G, Specchia G et al; CYTO-PV Collaborative Group (2013) Cardiovascular events and intensity of treatment in polycythemia vera. N Engl J Med 368:22–33PubMedCrossRef Marchioli R, Finazzi G, Specchia G et al; CYTO-PV Collaborative Group (2013) Cardiovascular events and intensity of treatment in polycythemia vera. N Engl J Med 368:22–33PubMedCrossRef
53.
54.
go back to reference Chen H, Angerer JI, Napoleone M et al (2013) Hematocrit and flow rate regulate the adhesion of platelets to von Willebrand factor. Biomicrofluidics 7:64113PubMedCrossRef Chen H, Angerer JI, Napoleone M et al (2013) Hematocrit and flow rate regulate the adhesion of platelets to von Willebrand factor. Biomicrofluidics 7:64113PubMedCrossRef
55.
go back to reference Nuyttens BP, Thijs T, Deckmyn H, Broos K (2011) Platelet adhesion to collagen. Thromb Res 127(Suppl. 2):S26–S29PubMedCrossRef Nuyttens BP, Thijs T, Deckmyn H, Broos K (2011) Platelet adhesion to collagen. Thromb Res 127(Suppl. 2):S26–S29PubMedCrossRef
56.
go back to reference Holme PA, Orvim U, Hamers MJ et al (1997) Shear induced platelet activation and platelet microparticle formation at blood flow conditions as in arteries with a severe stenosis. Arterioscler Thromb Vasc Biol 17:646–653PubMedCrossRef Holme PA, Orvim U, Hamers MJ et al (1997) Shear induced platelet activation and platelet microparticle formation at blood flow conditions as in arteries with a severe stenosis. Arterioscler Thromb Vasc Biol 17:646–653PubMedCrossRef
57.
go back to reference De Grandis M, Cambot M, Wautier MP et al (2013) JAK2V617F activates Lu/BCAM-mediated red cell adhesion in polycythemia vera through an EpoR-independent Rap1/Akt pathway. Blood 121:658–665PubMedCrossRef De Grandis M, Cambot M, Wautier MP et al (2013) JAK2V617F activates Lu/BCAM-mediated red cell adhesion in polycythemia vera through an EpoR-independent Rap1/Akt pathway. Blood 121:658–665PubMedCrossRef
58.
go back to reference Tefferi A, Gangat N, Wolanskyj AP (2006) Management of extreme thrombocytosis in otherwise low-risk essential thrombocythemia; does number matter? Blood 108:2493–2494PubMedCrossRef Tefferi A, Gangat N, Wolanskyj AP (2006) Management of extreme thrombocytosis in otherwise low-risk essential thrombocythemia; does number matter? Blood 108:2493–2494PubMedCrossRef
59.
go back to reference Castaman G, Lattuada A, Ruggeri M, Tosetto A, Mannucci PM, Rodeghiero F (1995) Platelet von Willebrand factor abnormalities in myeloproliferative syndromes. Am J Hematol 49:289–293PubMedCrossRef Castaman G, Lattuada A, Ruggeri M, Tosetto A, Mannucci PM, Rodeghiero F (1995) Platelet von Willebrand factor abnormalities in myeloproliferative syndromes. Am J Hematol 49:289–293PubMedCrossRef
60.
go back to reference Kissova J, Bulikova A, Ovesna P, Bourkova L, Penka M (2014) Increased mean platelet volume and immature platelet fraction as potential predictors of thrombotic complications in BCR/ABL-negative myeloproliferative neoplasms. Int J Hematol 100:429–436PubMedCrossRef Kissova J, Bulikova A, Ovesna P, Bourkova L, Penka M (2014) Increased mean platelet volume and immature platelet fraction as potential predictors of thrombotic complications in BCR/ABL-negative myeloproliferative neoplasms. Int J Hematol 100:429–436PubMedCrossRef
61.
go back to reference Gugliotta L, Iurlo A, Gugliotta G, Tieghi A et al (2016) Unbiased pro-thrombotic features at diagnosis in 977 thrombocythemic patients with Philadelphia-negative chronic myeloproliferative neoplasms. Leuk Res 46:18–25PubMedCrossRef Gugliotta L, Iurlo A, Gugliotta G, Tieghi A et al (2016) Unbiased pro-thrombotic features at diagnosis in 977 thrombocythemic patients with Philadelphia-negative chronic myeloproliferative neoplasms. Leuk Res 46:18–25PubMedCrossRef
62.
go back to reference Michiels JJ, Berneman Z, Schroyens W et al (2006) The paradox of platelet activation and impaired function: platelet-von Willebrand factor interactions, and the etiology of thrombotic and hemorrhagic manifestations in essential thrombocythemia and polycythemia vera. Semin Thromb Hemost 32:589–604PubMedCrossRef Michiels JJ, Berneman Z, Schroyens W et al (2006) The paradox of platelet activation and impaired function: platelet-von Willebrand factor interactions, and the etiology of thrombotic and hemorrhagic manifestations in essential thrombocythemia and polycythemia vera. Semin Thromb Hemost 32:589–604PubMedCrossRef
63.
go back to reference Regev A, Stark P, Blickstein D et al (1997) Thrombotic complications in essential thrombocythemia with relatively low platelet counts. Am J Hematol 56:168–172PubMedCrossRef Regev A, Stark P, Blickstein D et al (1997) Thrombotic complications in essential thrombocythemia with relatively low platelet counts. Am J Hematol 56:168–172PubMedCrossRef
64.
go back to reference Buxhofer-Ausch V, Steurer M, Sormann S et al (2016) Influence of platelet and white blood cell counts on major thrombosis—analysis from a patient registry in essential thrombocythemia. Eur J Haematol 97:511–516PubMedCrossRef Buxhofer-Ausch V, Steurer M, Sormann S et al (2016) Influence of platelet and white blood cell counts on major thrombosis—analysis from a patient registry in essential thrombocythemia. Eur J Haematol 97:511–516PubMedCrossRef
65.
go back to reference Augello C, Cattaneo D, Bucelli C et al (2016) CD18 promoter methylation is associated with a higher risk of thrombotic complications in primary myelofibrosis. Ann Hematol 95:1965–1969PubMedCrossRef Augello C, Cattaneo D, Bucelli C et al (2016) CD18 promoter methylation is associated with a higher risk of thrombotic complications in primary myelofibrosis. Ann Hematol 95:1965–1969PubMedCrossRef
66.
go back to reference Aird WC (2012) Endothelial cell heterogeneity. Cold Spring Harb Perspect Med 2:1–13CrossRef Aird WC (2012) Endothelial cell heterogeneity. Cold Spring Harb Perspect Med 2:1–13CrossRef
67.
go back to reference Cines DB, Pollak ES, Buck CA et al (1998) Endothelial cells in physiology and in the pathophysiology of vascular disorders. Blood 91:3527–3561PubMed Cines DB, Pollak ES, Buck CA et al (1998) Endothelial cells in physiology and in the pathophysiology of vascular disorders. Blood 91:3527–3561PubMed
68.
go back to reference Bevilacqua MP (1993) Endothelial-leukocyte adhesion molecules. Ann Rev Immunol 11:767–804CrossRef Bevilacqua MP (1993) Endothelial-leukocyte adhesion molecules. Ann Rev Immunol 11:767–804CrossRef
69.
go back to reference Mutunga M, Fulton B, Bullock R et al (2001) Circulating endothelial cells in patients with septic shock. Am J Respir Crit Care Med 163:195–200PubMedCrossRef Mutunga M, Fulton B, Bullock R et al (2001) Circulating endothelial cells in patients with septic shock. Am J Respir Crit Care Med 163:195–200PubMedCrossRef
70.
go back to reference Woywodt A, Scheer J, Hambach L et al (2004) Circulating endothelial cells as a marker of endothelial damage in allogeneic hematopoietic stem cell transplantation. Blood 103:3603–3605PubMedCrossRef Woywodt A, Scheer J, Hambach L et al (2004) Circulating endothelial cells as a marker of endothelial damage in allogeneic hematopoietic stem cell transplantation. Blood 103:3603–3605PubMedCrossRef
71.
go back to reference Mancuso P, Burlini A, Pruneri G et al (2001) Resting and activated endothelial cells are increased in the peripheral blood of cancer patients. Blood 97:3658–3661PubMedCrossRef Mancuso P, Burlini A, Pruneri G et al (2001) Resting and activated endothelial cells are increased in the peripheral blood of cancer patients. Blood 97:3658–3661PubMedCrossRef
72.
go back to reference Wassmann S, Werner N, Czech T, Nickenig G (2006) Improvement of endothelial function by systemic transfusion of vascular progenitor cells. Circ Res 99:e74–e83PubMedCrossRef Wassmann S, Werner N, Czech T, Nickenig G (2006) Improvement of endothelial function by systemic transfusion of vascular progenitor cells. Circ Res 99:e74–e83PubMedCrossRef
73.
go back to reference Torres C, Fonseca AM, Leander M et al (2013) Circulating endothelial cells in patients with venous thromboembolism and myeloproliferative neoplasms. PLoS ONE 8:e81574PubMedPubMedCentralCrossRef Torres C, Fonseca AM, Leander M et al (2013) Circulating endothelial cells in patients with venous thromboembolism and myeloproliferative neoplasms. PLoS ONE 8:e81574PubMedPubMedCentralCrossRef
74.
go back to reference Rosti V, Bonetti E, Bergamaschi G et al; AGIMM Investigators (2010) High frequency of endothelial colony forming cells marks a nonactive myeloproliferative neoplasm with high risk of splanchnic vein thrombosis. PLoS ONE 5:e15277PubMedPubMedCentralCrossRef Rosti V, Bonetti E, Bergamaschi G et al; AGIMM Investigators (2010) High frequency of endothelial colony forming cells marks a nonactive myeloproliferative neoplasm with high risk of splanchnic vein thrombosis. PLoS ONE 5:e15277PubMedPubMedCentralCrossRef
75.
go back to reference Yoder MC, Mead LE, Prater D et al (2007) Redefining endothelial progenitor cells via clonal analysis and hematopoietic stem/progenitor cell principals. Blood 109:1801–1809PubMedPubMedCentralCrossRef Yoder MC, Mead LE, Prater D et al (2007) Redefining endothelial progenitor cells via clonal analysis and hematopoietic stem/progenitor cell principals. Blood 109:1801–1809PubMedPubMedCentralCrossRef
76.
go back to reference Teofili L, Martini M, Iachininoto MG et al (2011) Endothelial progenitor cells are clonal and exhibit the JAK2(V617F) mutation in a subset of thrombotic patients with Ph-negative myeloproliferative neoplasms. Blood 117:2700–2707PubMedCrossRef Teofili L, Martini M, Iachininoto MG et al (2011) Endothelial progenitor cells are clonal and exhibit the JAK2(V617F) mutation in a subset of thrombotic patients with Ph-negative myeloproliferative neoplasms. Blood 117:2700–2707PubMedCrossRef
77.
go back to reference Fleischman AG, Aichberger KJ, Luty SB et al (2011) TNFα facilitates clonal expansion of JAK2V617F positive cells in myeloproliferative neoplasms. Blood 118:6392–6398PubMedPubMedCentralCrossRef Fleischman AG, Aichberger KJ, Luty SB et al (2011) TNFα facilitates clonal expansion of JAK2V617F positive cells in myeloproliferative neoplasms. Blood 118:6392–6398PubMedPubMedCentralCrossRef
78.
go back to reference Kleppe M, Kwak M, Koppikar P et al (2015) JAK-STAT pathway activation in malignant and nonmalignant cells contributes to MPN pathogenesis and therapeutic response. Cancer Discov 5:316–331PubMedPubMedCentralCrossRef Kleppe M, Kwak M, Koppikar P et al (2015) JAK-STAT pathway activation in malignant and nonmalignant cells contributes to MPN pathogenesis and therapeutic response. Cancer Discov 5:316–331PubMedPubMedCentralCrossRef
79.
go back to reference Ait-Oufella H, Taleb S, Mallat Z, Tedgui A (2011) Recent advances on the role of cytokines in atherosclerosis. Arterioscler Thromb Vasc Biol 31:969–979PubMedCrossRef Ait-Oufella H, Taleb S, Mallat Z, Tedgui A (2011) Recent advances on the role of cytokines in atherosclerosis. Arterioscler Thromb Vasc Biol 31:969–979PubMedCrossRef
80.
81.
go back to reference Barbui T, Carobbio A, Finazzi G et al; AGIMM and IIC Investigators (2011) Inflammation and thrombosis in essential thrombocythemia and polycythemia vera: different role of C-reactive protein and pentraxin 3. Haematologica 96:315–318PubMedCrossRef Barbui T, Carobbio A, Finazzi G et al; AGIMM and IIC Investigators (2011) Inflammation and thrombosis in essential thrombocythemia and polycythemia vera: different role of C-reactive protein and pentraxin 3. Haematologica 96:315–318PubMedCrossRef
82.
go back to reference Eisenreich A, Bogdanov VY, Zakrzewicz A et al (2009) Cdc2-like kinases and DNA topoisomerase regulate alternative splicing of tissue factor in human endothelial cells. Circ Res 104:589–599PubMedCrossRef Eisenreich A, Bogdanov VY, Zakrzewicz A et al (2009) Cdc2-like kinases and DNA topoisomerase regulate alternative splicing of tissue factor in human endothelial cells. Circ Res 104:589–599PubMedCrossRef
83.
go back to reference Chen Y, Wang J, Yao Y et al (2009) Crp regulates the expression and activity of tissue factor as well as tissue factor pathway inhibitor via nf-kappab and erk ½ mapk pathway. FEBS Lett 583:2811–2818PubMedCrossRef Chen Y, Wang J, Yao Y et al (2009) Crp regulates the expression and activity of tissue factor as well as tissue factor pathway inhibitor via nf-kappab and erk ½ mapk pathway. FEBS Lett 583:2811–2818PubMedCrossRef
84.
go back to reference Steffel J, Akhmedov A, Greutert H, Luscher TF, Tanner FC (2005) Histamine induces tissue factor expression: implications for acute coronary syndromes. Circulation 112:341–349PubMedCrossRef Steffel J, Akhmedov A, Greutert H, Luscher TF, Tanner FC (2005) Histamine induces tissue factor expression: implications for acute coronary syndromes. Circulation 112:341–349PubMedCrossRef
85.
go back to reference Kawano H, Tsuji H, Nishimura H, Kimura S, Yano S, Ukimura N et al (2001) Serotonin induces the expression of tissue factor and plasminogen activator inhibitor-1 in cultured rat aortic endothelial cells. Blood 97:1697–1702PubMedCrossRef Kawano H, Tsuji H, Nishimura H, Kimura S, Yano S, Ukimura N et al (2001) Serotonin induces the expression of tissue factor and plasminogen activator inhibitor-1 in cultured rat aortic endothelial cells. Blood 97:1697–1702PubMedCrossRef
86.
go back to reference Rajnics P, Kellner Á, Karádi É et al (2016) Increased Lipocalin 2 level may have important role in thrombotic events in patients with polycythemia vera and essential thrombocythemia. Leuk Res 48:101–106PubMedCrossRef Rajnics P, Kellner Á, Karádi É et al (2016) Increased Lipocalin 2 level may have important role in thrombotic events in patients with polycythemia vera and essential thrombocythemia. Leuk Res 48:101–106PubMedCrossRef
87.
go back to reference Dignat George F (2008) Microparticles in vascular diseases. Thromb Res 122:555–559 Dignat George F (2008) Microparticles in vascular diseases. Thromb Res 122:555–559
88.
go back to reference Nomura S, Ozaki Y, Ikeda Y (2008) Function and role of microparticles in various clinical settings. Thromb Res 123:8–23PubMedCrossRef Nomura S, Ozaki Y, Ikeda Y (2008) Function and role of microparticles in various clinical settings. Thromb Res 123:8–23PubMedCrossRef
89.
go back to reference Burnier L, Fontana P, Kwak BR, Angelillo-Scherrer A (2009) Cell-derived microparticles in haemostasis and vascular medicine. Thromb Haemost 101:439–451PubMed Burnier L, Fontana P, Kwak BR, Angelillo-Scherrer A (2009) Cell-derived microparticles in haemostasis and vascular medicine. Thromb Haemost 101:439–451PubMed
90.
go back to reference Trappenburg MC, van Schilfgaarde M, Marchetti M et al (2009) Elevated procoagulant microparticles expressing endothelial and platelet markers in essential thrombocytopenia. Haematologica 94:911–918PubMedPubMedCentralCrossRef Trappenburg MC, van Schilfgaarde M, Marchetti M et al (2009) Elevated procoagulant microparticles expressing endothelial and platelet markers in essential thrombocytopenia. Haematologica 94:911–918PubMedPubMedCentralCrossRef
91.
go back to reference Taniguchi Y, Tanaka H, Luis EJ et al (2017) Elevated plasma levels of procoagulant microparticles are a novel risk factor for thrombosis in patients with myeloproliferative neoplasms. Int J Hematol 106:691–703PubMedCrossRef Taniguchi Y, Tanaka H, Luis EJ et al (2017) Elevated plasma levels of procoagulant microparticles are a novel risk factor for thrombosis in patients with myeloproliferative neoplasms. Int J Hematol 106:691–703PubMedCrossRef
92.
go back to reference Stein BL, McMahon B, Weiss I et al (2012) Tissue-factor bearing microparticles and thrombotic risk in the myeloproliferative neoplasms. ASH annual meeting abstracts 2012 [abstract 1145] Stein BL, McMahon B, Weiss I et al (2012) Tissue-factor bearing microparticles and thrombotic risk in the myeloproliferative neoplasms. ASH annual meeting abstracts 2012 [abstract 1145]
93.
go back to reference Marchetti M, Tartari CJ, Russo L et al (2014) Phospholipid-dependent procoagulant activity is highly expressed by circulating microparticles in patients with essential thrombocythemia. Am J Hematol 89:68–73PubMedCrossRef Marchetti M, Tartari CJ, Russo L et al (2014) Phospholipid-dependent procoagulant activity is highly expressed by circulating microparticles in patients with essential thrombocythemia. Am J Hematol 89:68–73PubMedCrossRef
94.
go back to reference Duchemin J, Ugo V, Ianotto JC, Lecucq L, Mercier B, Abgrall JF (2010) Increased circulating procoagulant activity and thrombin generation in patients with myeloproliferative neoplasms. Thromb Res 126:238–242PubMedCrossRef Duchemin J, Ugo V, Ianotto JC, Lecucq L, Mercier B, Abgrall JF (2010) Increased circulating procoagulant activity and thrombin generation in patients with myeloproliferative neoplasms. Thromb Res 126:238–242PubMedCrossRef
95.
go back to reference Han Y, Zhao S, ZhangW, Cen J, Zhang W, Qiu H et al (2013) Clinical significance of circulating microparticles in Ph-myeloproliferative neoplasms (MPN). Blood (ASH Annual Meeting Abstracts), 122; 2013 [abstract 2368] Han Y, Zhao S, ZhangW, Cen J, Zhang W, Qiu H et al (2013) Clinical significance of circulating microparticles in Ph-myeloproliferative neoplasms (MPN). Blood (ASH Annual Meeting Abstracts), 122; 2013 [abstract 2368]
97.
go back to reference Key NS, Mackman N (2010) Tissue factor and its measurement in whole blood, plasma, and microparticles. Semin Thromb Hemost 36:865–875PubMedCrossRef Key NS, Mackman N (2010) Tissue factor and its measurement in whole blood, plasma, and microparticles. Semin Thromb Hemost 36:865–875PubMedCrossRef
98.
go back to reference Mackman N, Tilley RE, Key NS (2007) Role of the extrinsic pathway of blood coagulation in hemostasis and thrombosis. Arterioscler Thromb Vasc Biol 27:1687–1693PubMedCrossRef Mackman N, Tilley RE, Key NS (2007) Role of the extrinsic pathway of blood coagulation in hemostasis and thrombosis. Arterioscler Thromb Vasc Biol 27:1687–1693PubMedCrossRef
99.
go back to reference Van Der Meijden PEJ, Van Schilfgaarde M, Van Oerle R, Renn ET, Ten Cate H, Spronk HMH (2012) Platelet- and erythrocyte-derived microparticles trigger thrombin generation via factor XIIa. J Thromb Haemost 10:1355–1362CrossRef Van Der Meijden PEJ, Van Schilfgaarde M, Van Oerle R, Renn ET, Ten Cate H, Spronk HMH (2012) Platelet- and erythrocyte-derived microparticles trigger thrombin generation via factor XIIa. J Thromb Haemost 10:1355–1362CrossRef
100.
go back to reference Furie B, Furie BC (2004) Role of platelet P-selectin and microparticle PSGL-1 in thrombus formation. Trends Mol Med 10:171–178PubMedCrossRef Furie B, Furie BC (2004) Role of platelet P-selectin and microparticle PSGL-1 in thrombus formation. Trends Mol Med 10:171–178PubMedCrossRef
101.
go back to reference Jensen MK, de Nully Brown P, Lund BV, Nielsen OJ, Hasselbalch HC (2000) Increased platelet activation and abnormal membrane glycoprotein content and redistribution in myeloproliferative disorders. Br J Haematol 110:116–124PubMedCrossRef Jensen MK, de Nully Brown P, Lund BV, Nielsen OJ, Hasselbalch HC (2000) Increased platelet activation and abnormal membrane glycoprotein content and redistribution in myeloproliferative disorders. Br J Haematol 110:116–124PubMedCrossRef
103.
go back to reference Arellano-Rodrigo E, Alvarez-Larrán A, Reverter JC, Villamor N, Colomer D, Cervantes F (2006) Increased platelet and leukocyte activation as contributing mechanisms for thrombosis in essential thrombocythemia and correlation with the JAK2 mutational status. Haematologica 91:169–175PubMed Arellano-Rodrigo E, Alvarez-Larrán A, Reverter JC, Villamor N, Colomer D, Cervantes F (2006) Increased platelet and leukocyte activation as contributing mechanisms for thrombosis in essential thrombocythemia and correlation with the JAK2 mutational status. Haematologica 91:169–175PubMed
104.
go back to reference Panova-Noeva M, Marchetti M, Russo L et al (2013) ADP-induced platelet aggregation and thrombin generation are increased in Essential Thrombocythemia and Polycythemia Vera. Thromb Res 132:88–93PubMedCrossRef Panova-Noeva M, Marchetti M, Russo L et al (2013) ADP-induced platelet aggregation and thrombin generation are increased in Essential Thrombocythemia and Polycythemia Vera. Thromb Res 132:88–93PubMedCrossRef
105.
go back to reference Panova-Noeva M, Marchetti M, Spronk HM et al (2011) Platelet-induced thrombin generation by the calibrated automated thrombogram assay is increased in patients with essential thrombocythemia and polycythemia vera. Am J Hematol 86:337–342PubMedCrossRef Panova-Noeva M, Marchetti M, Spronk HM et al (2011) Platelet-induced thrombin generation by the calibrated automated thrombogram assay is increased in patients with essential thrombocythemia and polycythemia vera. Am J Hematol 86:337–342PubMedCrossRef
106.
go back to reference Gadomska G, Stankowska K, Boinska J, Bartoszewska-Kubiak A, Haus O, Rość D (2016) Activation of the tissue factor-dependent extrinsic pathway and its relation to JAK2 V617F mutation status in patients with essential thrombocythemia. Blood Coagul Fibrinolysis 27:817–821PubMedCrossRef Gadomska G, Stankowska K, Boinska J, Bartoszewska-Kubiak A, Haus O, Rość D (2016) Activation of the tissue factor-dependent extrinsic pathway and its relation to JAK2 V617F mutation status in patients with essential thrombocythemia. Blood Coagul Fibrinolysis 27:817–821PubMedCrossRef
108.
go back to reference Presseizen K, Friedman Z, Shapiro H, Radnay J, Ellis MH (2002) Phosphatidylserine expression on the platelet membrane of patients with myeloproliferative disorders and its effect on platelet-dependent thrombin formation. Clin Appl Thromb Hemost 8:33–39PubMedCrossRef Presseizen K, Friedman Z, Shapiro H, Radnay J, Ellis MH (2002) Phosphatidylserine expression on the platelet membrane of patients with myeloproliferative disorders and its effect on platelet-dependent thrombin formation. Clin Appl Thromb Hemost 8:33–39PubMedCrossRef
109.
go back to reference Marchetti M, Castoldi E, Spronk HM et al (2008) Thrombin generation and activated protein C resistance in patients with essential thrombocythemia and polycythemia vera. Blood 112:4061–4068PubMedCrossRef Marchetti M, Castoldi E, Spronk HM et al (2008) Thrombin generation and activated protein C resistance in patients with essential thrombocythemia and polycythemia vera. Blood 112:4061–4068PubMedCrossRef
110.
go back to reference Arellano-Rodrigo E, Alvarez-Larrán A, Reverter JC et al (2009) Platelet turnover, coagulation factors, and soluble markers of platelet and endothelial activation in essential thrombocythemia: relationship with thrombosis occurrence and JAK2 V617F allele burden. Am J Hematol 84:102–108PubMedCrossRef Arellano-Rodrigo E, Alvarez-Larrán A, Reverter JC et al (2009) Platelet turnover, coagulation factors, and soluble markers of platelet and endothelial activation in essential thrombocythemia: relationship with thrombosis occurrence and JAK2 V617F allele burden. Am J Hematol 84:102–108PubMedCrossRef
111.
go back to reference Smalberg JH, Arends LR, Valla DC, Kiladjian JJ, Janssen HL, Leebeek FW (2012) Myeloproliferative neoplasms in Budd-Chiari syndrome and portal vein thrombosis: a meta-analysis. Blood 120:4921–4928PubMedCrossRef Smalberg JH, Arends LR, Valla DC, Kiladjian JJ, Janssen HL, Leebeek FW (2012) Myeloproliferative neoplasms in Budd-Chiari syndrome and portal vein thrombosis: a meta-analysis. Blood 120:4921–4928PubMedCrossRef
112.
go back to reference Dentali F, Squizzato A, Brivio L et al (2009) JAK2V617F mutation for the early diagnosis of Ph- myeloproliferative neoplasms in patients with venous thromboembolism: a meta-analysis. Blood 113:5617–5623PubMedCrossRef Dentali F, Squizzato A, Brivio L et al (2009) JAK2V617F mutation for the early diagnosis of Ph- myeloproliferative neoplasms in patients with venous thromboembolism: a meta-analysis. Blood 113:5617–5623PubMedCrossRef
113.
go back to reference De Stefano V, Za T, Rossi E et al; GIMEMA Chronic Myeloproliferative Neoplasms Working Party (2010) Increased risk of recurrent thrombosis in patients with essential thrombocythemia carrying the homozygous JAK2 V617Fmutation. Ann Hematol 89:141–146PubMedCrossRef De Stefano V, Za T, Rossi E et al; GIMEMA Chronic Myeloproliferative Neoplasms Working Party (2010) Increased risk of recurrent thrombosis in patients with essential thrombocythemia carrying the homozygous JAK2 V617Fmutation. Ann Hematol 89:141–146PubMedCrossRef
114.
go back to reference Vannucchi AM, Antonioli E, Guglielmelli P et al (2007) Clinical profile of homozygous JAK2V617F mutation in patients with polycythemia vera or essential thrombocythemia. Blood 110:840–846PubMedCrossRef Vannucchi AM, Antonioli E, Guglielmelli P et al (2007) Clinical profile of homozygous JAK2V617F mutation in patients with polycythemia vera or essential thrombocythemia. Blood 110:840–846PubMedCrossRef
115.
go back to reference Bertozzi I, Bogoni G, Biagetti G et al (2017) Thromboses and hemorrhages are common in MPN patients with high JAK2V617F allele burden. Ann Hematol 96:1297–1302PubMedCrossRef Bertozzi I, Bogoni G, Biagetti G et al (2017) Thromboses and hemorrhages are common in MPN patients with high JAK2V617F allele burden. Ann Hematol 96:1297–1302PubMedCrossRef
116.
go back to reference Panova-Noeva M, Marchetti M, Buoro S et al (2011) JAK2V617F mutation and hydroxyurea treatment as determinants of immature platelet parameters in essential thrombocythemia and polycythemia vera patients. Blood 118:2599–2601PubMedCrossRef Panova-Noeva M, Marchetti M, Buoro S et al (2011) JAK2V617F mutation and hydroxyurea treatment as determinants of immature platelet parameters in essential thrombocythemia and polycythemia vera patients. Blood 118:2599–2601PubMedCrossRef
117.
go back to reference Kogan I, Chap D, Hoffman R et al (2016) JAK-2 V617F mutation increases heparanase procoagulant activity. Thromb Haemost 115:73–80PubMedCrossRef Kogan I, Chap D, Hoffman R et al (2016) JAK-2 V617F mutation increases heparanase procoagulant activity. Thromb Haemost 115:73–80PubMedCrossRef
118.
go back to reference Rosti V, Villani L, Riboni R et al (2013) Spleen endothelial cells from patients with myelofibrosis harbor the JAK2V617F mutation. Blood 121:360–368PubMedCrossRef Rosti V, Villani L, Riboni R et al (2013) Spleen endothelial cells from patients with myelofibrosis harbor the JAK2V617F mutation. Blood 121:360–368PubMedCrossRef
119.
go back to reference Verstovsek S, Kantarjian H, Mesa RA et al (2010) Safety and efficacy of INCB018424, a JAK1 and JAK2 inhibitor, in myelofibrosis. N Engl J Med 363:1117–1127PubMedPubMedCentralCrossRef Verstovsek S, Kantarjian H, Mesa RA et al (2010) Safety and efficacy of INCB018424, a JAK1 and JAK2 inhibitor, in myelofibrosis. N Engl J Med 363:1117–1127PubMedPubMedCentralCrossRef
120.
go back to reference Verstovsek S, Passamonti F, Rambaldi A et al (2012) Long-term efficacy and safety results from a phase II study of Ruxolitinib in patients with polycythemia vera. ASH Annual Meeting. Abstract 120(21):804 Verstovsek S, Passamonti F, Rambaldi A et al (2012) Long-term efficacy and safety results from a phase II study of Ruxolitinib in patients with polycythemia vera. ASH Annual Meeting. Abstract 120(21):804
121.
go back to reference Rumi E, Pietra D, Ferretti V et al (2014) JAK2 or CALR mutation status defines subtypes of essential thrombocythemia with substantially different clinical course and outcomes. Blood 123:1544–1551PubMedPubMedCentralCrossRef Rumi E, Pietra D, Ferretti V et al (2014) JAK2 or CALR mutation status defines subtypes of essential thrombocythemia with substantially different clinical course and outcomes. Blood 123:1544–1551PubMedPubMedCentralCrossRef
122.
go back to reference Rotunno G, Mannarelli C, Guglielmelli P et al (2014) Impact of calreticulin mutations on clinical and hematological phenotype and outcome in essential thrombocythemia. Blood 123:1552–1555PubMedCrossRef Rotunno G, Mannarelli C, Guglielmelli P et al (2014) Impact of calreticulin mutations on clinical and hematological phenotype and outcome in essential thrombocythemia. Blood 123:1552–1555PubMedCrossRef
123.
go back to reference Finazzi G, Carobbio A, Guglielmelli P et al (2014) Calreticulin mutation does not modify the IPSET score for predicting the risk of thrombosis among 1150 patients with essential thrombocythemia. Blood 124:2611–2612PubMedCrossRef Finazzi G, Carobbio A, Guglielmelli P et al (2014) Calreticulin mutation does not modify the IPSET score for predicting the risk of thrombosis among 1150 patients with essential thrombocythemia. Blood 124:2611–2612PubMedCrossRef
124.
125.
go back to reference Finazzi MC, Carobbio A, Cervantes F et al (2015) CALR mutation, MPL mutation, and triple negativity identify patients with the lowest vascular risk in primary myelofibrosis. Leukemia 29:1209–1210PubMedCrossRef Finazzi MC, Carobbio A, Cervantes F et al (2015) CALR mutation, MPL mutation, and triple negativity identify patients with the lowest vascular risk in primary myelofibrosis. Leukemia 29:1209–1210PubMedCrossRef
Metadata
Title
Thrombosis in Philadelphia negative classical myeloproliferative neoplasms: a narrative review on epidemiology, risk assessment, and pathophysiologic mechanisms
Authors
Somedeb Ball
Kyaw Zin Thein
Abhishek Maiti
Kenneth Nugent
Publication date
01-05-2018
Publisher
Springer US
Published in
Journal of Thrombosis and Thrombolysis / Issue 4/2018
Print ISSN: 0929-5305
Electronic ISSN: 1573-742X
DOI
https://doi.org/10.1007/s11239-018-1623-4

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