The myeloproliferative disorders

New England Journal of Medicine

Volumn 355, Issue 23, 2006, Pages 2452-2466

  Campbell, Peter J ^a   Green, Anthony R ^a  

^a UNIVERSITY OF CAMBRIDGE   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

ERYTHROPOIETIN; JANUS KINASE 2; JANUS KINASE 2 INHIBITOR; JANUS KINASE INHIBITOR;

CELL DIFFERENTIATION; CLINICAL FEATURE; CLINICAL TRIAL; DIAGNOSTIC PROCEDURE; DISEASE CLASSIFICATION; DISEASE COURSE; DRUG MECHANISM; GENE DELETION; GENE MUTATION; GENETIC ANALYSIS; GENETIC MARKER; HOMOZYGOSITY; HUMAN; MYELOPROLIFERATIVE DISORDER; NONHUMAN; PATHOGENESIS; PATHOPHYSIOLOGY; PRIORITY JOURNAL; REVIEW; STEM CELL;

GENETIC MARKERS; HUMANS; JANUS KINASE 2; MULTIPOTENT STEM CELLS; MUTATION; MYELOPROLIFERATIVE DISORDERS; SIGNAL TRANSDUCTION;

EID: 33845436745     PISSN: 00284793     EISSN: 15334406     Source Type: Journal    
DOI: 10.1056/NEJMra063728     Document Type: Review

References (119)

1. Goldman JM, Melo JV. Chronic myeloid leukemia - advances in biology and new approaches to treatment. N Engl J Med 2003;349:1451-64.
2. Pierre R, Imbert M, Thiele J, Vardiman JW, Brunning RD, Flandrin G. Chronic myeloproliferative diseases. In: Jaffe ES, Harris NL, Stein H, Vardiman JW, eds. World Health Organization classification of tumours: pathology and genetics of tumours of haemopoietic and lymphoid tissues. Lyon, France: IARC Press, 2001:61-73.
3. Johansson P, Kutti J, Andreasson B, et al. Trends in the incidence of chronic Philadelphia chromosome negative (Ph-) myeloproliferative disorders in the city of Goteborg, Sweden, during 1983-99. J Intern Med 2004;256:161-5.
4. Vaquez H. Sur une forme speciale de cyanose s'accompagnant d'hyperglobulie excessive et persistante. C R Soc Biol (Paris) 1892;44:384-8.
5. Dameshek W. Some speculations on the myeloproliferative syndromes. Blood 1951;6:372-5.
6. Baxter EJ, Scott LM, Campbell PJ, et al. Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders. Lancet 2005;365:1054-6. [Erratum, Lancet 2005;366:122.]
7. James C, Ugo V, Le Couedic JP, et al. A unique clonal JAK2 mutation leading to constitutive signaling causes polycythaemia vera. Nature 2005;434:1144-8.
8. Levine RL, Wadleigh M, Cools J, et al. Activating mutation in the tyrosine kinase JAK2 in polycythemia vera, essential thrombocythemia, and myeloid metaplasia with myelofibrosis. Cancer Cell 2005;7:387-97.
9. Kralovics R, Passamonti F, Buser AS, et al. A gain-of-function mutation of JAK2 in myeloproliferative disorders. N Engl J Med 2005;352:1779-90.
10. Prchal JF, Axelrad AA. Bone-marrow responses in polycythemia vera. N Engl J Med 1974;290:1382.
11. Adamson JW, Fialkow PJ, Murphy S, Prchal JF, Steinmann L. Polycythemia vera: stem-cell and probable clonal origin of the disease. N Engl J Med 1976;295:913-6.
12. Jamieson CH, Gotlib J, Durocher JA, et al. The JAK2 V617F mutation occurs in hematopoietic stem cells in polycythemia vera and predisposes toward erythroid differentiation. Proc Natl Acad Sci U S A 2006;103:6224-9.
13. Bartram CR, de Klein A, Hagemeijer A, et al. Translocation of c-ab1 oncogene correlates with the presence of a Philadelphia chromosome in chronic myelocytic leukaemia. Nature 1983;306:277-80.
14. Lugo TG, Pendergast AM, Muller AJ, Witte ON. Tyrosine kinase activity and transformation potency of bcr-abl oncogene products. Science 1990;247:1079-82.
15. Buttner C, Henz BM, Welker P, Sepp NT, Grabbe J. Identification of activating c-kit mutations in adult-, but not in childhood-onset indolent mastocytosis: a possible explanation for divergent clinical behavior. J Invest Dermatol 1998;111:1227-31.
16. Golub TR, Barker GF, Lovett M, Gilliland DG. Fusion of PDGF receptor beta to a novel ets-like gene, tel, in chronic myelomonocytic leukemia with t(5;12) chromosomal translocation. Cell 1994;77:307-16.
17. Xiao S, Nalabolu SR, Aster JC, et al. FGFR1 is fused with a novel zinc-finger gene, ZNF198, in the t(8;13) leukaemia/lymphoma syndrome. Nat Genet 1998;18:84-7.
18. Cools J, DeAngelo DJ, Gotlib J, et al. A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. N Engl J Med 2003;348:1201-14.
19. Zhao R, Xing S, Li Z, et al. Identification of an acquired JAK2 mutation in polycythemia vera. J Biol Chem 2005;280:22788-92.
20. Kralovics R, Guan Y, Prchal JT. Acquired uniparental disomy of chromosome 9p is a frequent stem cell defect in polycythemia vera. Exp Hematol 2002;30:229-36.
21. Roder S, Steimle C, Meinhardt G, Pahl HL. STAT3 is constitutively active in some patients with polycythemia rubra vera. Exp Hematol 2001;29:694-702.
22. Ugo V, Marzac C, Teyssandier I, et al. Multiple signaling pathways are involved in erythropoietin-independent differentiation of erythroid progenitors in polycythemia vera. Exp Hematol 2004;32:179-87.
23. Heuck G. Falle von Leukaemie mit eigenthumlichen Blut-resp. Knockenmarksbefund. Virchown Archiv 1879;78:475-81.
24. Epstein E, Goedel A. Haemorrhagische thrombocythamie bei vascularer schrumpfmilz. Virchov's Archiv Abteilung 1934;293:233-47.
25. Fialkow PJ, Faguet GB, Jacobson RJ, Vaidya K, Murphy S. Evidence that essential thrombocythemia is a clonal disorder with origin in a multipotent stem cell. Blood 1981;58:916-9.
26. Levine RL, Belisle C, Wadleigh M, et al. X-inactivation-based clonality analysis and quantitative JAK2V617F assessment reveals a strong association between clonality and JAK2V617F in PV but not ET/MMM, and identifies a subset of JAK2V617F-negative ET and MMM patients with clonal hematopoiesis. Blood 2006;107:4139-41.
27. Campbell PJ, Scott LM, Buck G, et al. Definition of subtypes of essential thrombocythaemia and relation to polycythaemia vera based on JAK2 V617F mutation status: a prospective study. Lancet 2005;366:1945-53.
28. Antonioli E, Guglielmelli P, Pancrazzi A, et al. Clinical implications of the JAK2 V617F mutation in essential thrombocythemia. Leukemia 2005;19:1847-9.
29. Campbell PJ, Griesshammer M, Dohner K, et al. V617F mutation in JAK2 is associated with poorer survival in idiopathic myelofibrosis. Blood 2006;107:2098-100.
30. Tefferi A, Lasho TL, Schwager SM, et al. The JAK2(V617F) tyrosine kinase mutation in myelofibrosis with myeloid metaplasia: lineage specificity and clinical correlates. Br J Haematol 2005;131:320-8.
31. Jones AV, Kreil S, Zoi K, et al. Widespread occurrence of the JAK2 V617F mutation in chronic myeloproliferative disorders. Blood 2005;106:2162-8.
32. Steensma DP, Dewald GW, Lasho TL, et al. The JAK2 V617F activating tyrosine kinase mutation is an infrequent event in both "atypical" myeloproliferative disorders and myelodysplastic syndromes. Blood 2005;106:1207-9.
33. Scott LM, Campbell PJ, Baxter EJ, et al. The V617F JAK2 mutation is uncommon in cancers and in myeloid malignancies other than the classic myeloproliferative disorders. Blood 2005;106:2920-1.
34. Levine RL, Loriaux M, Huntly BJ, et al. The JAK2V617F activating mutation occurs in chronic myelomonocytic leukemia and acute myeloid leukemia, but not in acute lymphoblastic leukemia or chronic lymphocytic leukemia. Blood 2005;106:3377-9.
35. Pikman Y, Lee BH, Mercher T, et al. MPLW515L is a novel somatic activating mutation in myelofibrosis with myeloid metaplasia. PLoS Med 2006;3:e270.
36. Pardanani AD, Levine RL, Lasho T, et al. MPL515 mutations in myeloproliferative and other myeloid disorders: a study of 1182 patients. Blood (in press).
37. Longley BJ Jr, Metcalfe DD, Tharp M, et al. Activating and dominant inactivating c-KIT catalytic domain mutations in distinct clinical forms of human mastocytosis. Proc Natl Acad Sci U S A 1999;96:1609-14.
38. Pardanani A, Brockman SR, Paternoster SF, et al. FIP1L1-PDGFRA fusion: prevalence and clinicopathologic correlates in 89 consecutive patients with moderate to severe eosinophilia. Blood 2004;104:3038-45.
39. Campbell PJ, Baxter EJ, Beer PA, et al. Mutation of JAK2 in the myeloproliferative disorders: timing, clonality studies, cytogenetic associations and role in leukemic transformation. Blood (in press).
40. Bench AJ, Nacheva EP, Champion KM, Green AR. Molecular genetics and cytogenetics of myeloproliferative disorders. Baillieres Clin Haematol 1998;11:819-48.
41. Bench AJ, Nacheva EP, Hood TL, et al. Chromosome 20 deletions in myeloid malignancies: reduction of the common deleted region, generation of a PAC/BAC contig and identification of candidate genes. Oncogene 2000;19:3902-13.
42. Kralovics R, Teo SS, Li S, et al. Acquisition of the V617F mutation of JAK2 is a late genetic event in a subset of patients with myeloproliferative disorders. Blood 2006;108:1377-80.
43. Silva M, Richard C, Benito A, Sanz C, Olalla I, Fernandez-Luna JL. Expression of Bcl-x in erythroid precursors from patients with polycythemia vera. N Engl J Med 1998;338:564-71.
44. Kieslinger M, Woldman I, Moriggl R, et al. Antiapoptotic activity of Stat5 required during terminal stages of myeloid differentiation. Genes Dev 2000;14:232-44.
45. Socolovsky M, Nam H, Fleming MD, Haase VH, Brugnara C, Lodish HF. Ineffective erythropoiesis in Stat5a(-/-)5b(-/-) mice due to decreased survival of early erythroblasts. Blood 2001;98:3261-73.
46. Kralovics R, Teo SS, Buser AS, et al. Altered gene expression in myeloproliferative disorders correlates with activation of signaling by the V617F mutation of Jak2. Blood 2005;106:3374-6.
47. Goerttler PS, Kreutz C, Donauer J, et al. Gene expression profiling in polycythaemia vera: overexpression of transcription factor NF-E2. Br J Haematol 2005;129:138-50.
48. Labbaye C, Valtieri M, Barberi T, et al. Differential expression and functional role of GATA-2, NF-E2, and GATA-1 in normal adult hematopoiesis. J Clin Invest 1995;95:2346-58.
49. Sayer MS, Tilbrook PA, Spadaccini A, et al. Ectopic expression of transcription factor NF-E2 alters the phenotype of erythroid and monoblastoid cells. J Biol Chem 2000;275:25292-8.
50. Goerttler PS, Steimle C, Marz E, et al. The Jak2V617F mutation, PRV-1 overexpression, and EEC formation define a similar cohort of MPD patients. Blood 2005;106:2862-4.
51. Klippel S, Strunck E, Busse CE, Behringer D, Pahl HL. Biochemical characterization of PRV-1, a novel hematopoietic cell surface receptor, which is overexpressed in polycythemia rubra vera. Blood 2002;100:2441-8.
52. Moliterno AR, Hankins WD, Spivak JL. Impaired expression of the thrombopoietin receptor by platelets from patients with polycythemia vera. N Engl J Med 1998;338:572-80.
53. Moliterno AR, Spivak JL. Posttranslational processing of the thrombopoietin receptor is impaired in polycythemia vera. Blood 1999;94:2555-61.
54. Komura E, Chagraoui H, Mansat de Mas V, et al. Spontaneous STAT5 activation induces growth factor independence in idiopathic myelofibrosis: possible relationship with FKBP51 overexpression. Exp Hematol 2003;31:622-30.
55. Sandberg EM, Wallace TA, Godeny MD, Vonderlinden D, Sayeski PP. Jak2 tyrosine kinase: a true jak of all trades? Cell Biochem Biophys 2004;41:207-32.
56. Kisseleva T, Bhattacharya S, Braunstein J, Schindler CW. Signaling through the JAK/STAT pathway, recent advances and future challenges. Gene 2002;285:1-24.
57. Neubauer H, Cumano A, Muller M, Wu H, Huffstadt U, Pfeffer K. Jak2 deficiency defines an essential developmental checkpoint in definitive hematopoiesis. Cell 1998;93:397-409.
58. Parganas E, Wang D, Stravopodis D, et al. Jak2 is essential for signaling through a variety of cytokine receptors. Cell 1998;93:385-95.
59. Huang LJ, Constantinescu SN, Lodish HF. The N-terminal domain of Janus kinase 2 is required for Golgi processing and cell surface expression of erythropoietin receptor. Mol Cell 2001;8:1327-38.
60. Remy I, Wilson IA, Michnick SW. Erythropoietin receptor activation by a ligandinduced conformation change. Science 1999;283:990-3.
61. Lu X, Gross AW, Lodish HF. Active conformation of the erythropoietin receptor: random and cysteine-scanning mutagenesis of the extracellular juxtamembrane and transmembrane domains. J Biol Chem 2006;281:7002-11.
62. Seubert N, Royer Y, Staerk J, et al. Active and inactive orientations of the transmembrane and cytosolic domains of the erythropoietin receptor dimer. Mol Cell 2003;12:1239-50.
63. Witthuhn BA, Quelle FW, Silvennoinen O, et al. JAK2 associates with the erythropoietin receptor and is tyrosine phosphorylated and activated following stimulation with erythropoietin. Cell 1993;74:227-36.
64. Wolanskyj AP, Lasho TL, Schwager SM, et al. JAK2 mutation in essential thrombocythaemia: clinical associations and long-term prognostic relevance. Br J Haematol 2005;131:208-13.
65. Scott LM, Scott MA, Campbell PJ, Green AR. Progenitors homozygous for the V617F JAK2 mutation occur in most patients with polycythemia vera but not essential thrombocythemia. Blood 2006;108:2435-7.
66. Jelinek J, Oki Y, Gharibyan V, et al. JAK2 mutation 1849G→T is rare in acute leukemias but can be found in CMML, Philadelphia chromosome-negative CML, and megakaryocytic leukemia. Blood 2005;106:3370-3.
67. Lee JW, Kim YG, Soung YH, et al. The JAK2 V617F mutation in de novo acute myelogenous leukemias. Oncogene 2006;25:1434-6.
68. Frohling S, Lipka DB, Kayser S, et al. Rare occurrence of the JAK2 V617F mutation in AML subtypes M5, M6, and M7. Blood 2006;107:1242-3.
69. Melzner I, Weniger MA, Menz CK, Moller P. Absence of the JAK2 V617F activating mutation in classical Hodgkin lymphoma and primary mediastinal B-cell lymphoma. Leukemia 2006;20:157-8.
70. Patel RK, Lea NC, Heneghan MA, et al. Prevalence of the activating JAK2 tyrosine kinase mutation V617F in the Budd-Chiari syndrome. Gastroenterology 2006;130:2031-8.
71. Saharinen P, Takaluoma K, Silvennoinen O. Regulation of the Jak2 tyrosine kinase by its pseudokinase domain. Mol Cell Biol 2000;20:3387-95.
72. Socolovsky M, Fallon AE, Wang S, Brugnara C, Lodish HF. Fetal anemia and apoptosis of red cell progenitors in Stat5a-/-5b-/- mice: a direct role for Stat5 in Bcl-X(L) induction. Cell 1999;98:181-91.
73. Myklebust JH, Blomhoff HK, Rusten LS, Stokke T, Smeland EB. Activation of phosphatidylinositol 3-kinase is important for erythropoietin-induced erythropoiesis from CD34(+) hematopoietic progenitor cells. Exp Hematol 2002;30:990-1000.
74. Staerk J, Lacout C, Sato T, Smith SO, Vainchenker W, Constantinescu SN. An amphipathic motif at the transmembrane-cytoplasmic junction prevents autonomous activation of the thrombopoietin receptor. Blood 2006;107:1864-71.
75. Wernig G, Mercher T, Okabe R, Levine RL, Lee BH, Gilliland DG. Expression of Jak2V617F causes a polycythemia vera-like disease with associated myelofibrosis in a murine bone marrow transplant model. Blood 2006;107:4274-81.
76. Lacout C, Pisani DF, Tulliez M, Gachelin FM, Vainchenker W, Villeval JL. JAK2V617F expression in murine hematopoietic cells leads to MPD mimicking human PV with secondary myelofibrosis. Blood 2006;108:1652-60.
77. Zaleskas VM, Krause DS, Lazarides K, et al. Molecular pathogenesis of polycythemia induced in mice by JAK2 V617F. Blood 2005;106:A116. abstract.
78. Bumm TGP, Elsea C, Wood LG, et al. JAK2 V617F mutation induces a myeloproliferative disorder in mice. Blood 2005;106:A116. abstract.
79. Gale RE. Evaluation of clonality in myeloid stem-cell disorders. Semin Hematol 1999;36:361-72.
80. Abkowitz JL, Taboada M, Shelton GH, Catlin SN, Guttorp P, Kiklevich JV. An X chromosome gene regulates hematopoietic stem cell kinetics. Proc Natl Acad Sci U S A 1998;95:3862-6.
81. Kiladjian JJ, Elkassar N, Cassinat B, et al. Essential thrombocythemias without V617F JAK2 mutation are clonal hematopoietic stem cell disorders. Leukemia 2006;20:1181-3.
82. Lasho TL, Mesa R, Gilliland DG, Tefferi A. Mutation studies in CD3+, CD19+ and CD34+ cell fractions in myeloproliferative disorders with homozygous JAK2 (V617F) in granulocytes. Br J Haematol 2005;130:797-9.
83. Jamieson CH, Ailles LE, Dylla SJ, et al. Granulocyte-macrophage progenitors as candidate leukemic stem cells in blast-crisis CML. N Engl J Med 2004;351:657-67.
84. Mesa RA, Powell H, Lasho T, Dewald G, McClure R, Tefferi A. JAK2(V617F) and leukemic transformation in myelofibrosis with myeloid metaplasia. Leuk Res 2006;30:1457-60.
85. Mesa RA, Powell H, Lasho T, DeWald GW, McClure R, Tefferi A. A longitudinal study of the JAK2(V617F) mutation in myelofibrosis with myeloid metaplasia: analysis at two time points. Haematologica 2006;91:415-6.
86. Bellanne-Chantelot C, Chaumarel I, Labopin M, et al. Genetic and clinical implications of the Val617Phe JAK2 mutation in 72 families with myeloproliferative disorders. Blood 2006;108:346-52.
87. Holt D, Dreimanis M, Pfeiffer M, Firgaira F, Morley A, Turner D. Interindividual variation in mitotic recombination. Am J Hum Genet 1999;65:1423-7.
88. Zeuner A, Pedini F, Signore M, et al. Increased death receptor resistance and FLIPshort expression in polycythemia vera erythroid precursor cells. Blood 2006;107:3495-502.
89. Walz C, Crowley BJ, Hudon HE, et al. Activated JAK2 with the V617F point mutation promotes G1/S-phase transition. J Biol Chem 2006;281:18177-83.
90. Staerk J, Kallin A, Demoulin JB, Vainchenker W, Constantinescu SN. JAK1 and Tyk2 activation by the homologous polycythemia vera JAK2 V617F mutation: crosstalk with IGF1 receptor. J Biol Chem 2005;280:41893-9.
91. Royer Y, Staerk J, Costuleanu M, Courtoy PJ, Constantinescu SN. Janus kinases affect thrombopoietin receptor cell surface localization and stability. J Biol Chem 2005;280:27251-61.
92. Vainchenker W, Constantinescu SN. A unique activating mutation in JAK2 (V617F) is at the origin of polycythemia vera and allows a new classification of myeloproliferative diseases. Hematology Am Soc Hematol Educ Program 2005;195-200.
93. Lu X, Levine R, Tong W, et al. Expression of a homodimeric type I cytokine receptor is required for JAK2V617F-mediated transformation. Proc Natl Acad Sci U S A 2005;102:18962-7.
94. Spivak JL. Polycythemia vera: myths, mechanisms, and management. Blood 2002;100:4272-90.
95. Cervantes F, Alvarez-Larran A, Talarn C, Gomez M, Montserrat E. Myelofibrosis with myeloid metaplasia following essential thrombocythaemia: actuarial probability, presenting characteristics and evolution in a series of 195 patients. Br J Haematol 2002;118:786-90.
96. Thiele J, Kvasnicka HM, Zankovich R, Diehl V. Relevance of bone marrow features in the differential diagnosis between essential thrombocythemia and early stage idiopathic myelofibrosis. Haematologica 2000;85:1126-34.
97. Tefferi A. Myelofibrosis with myeloid metaplasia. N Engl J Med 2000;342:1255-65.
98. Tefferi A. Idem. Pathogenesis of myelofibrosis with myeloid metaplasia. J Clin Oncol 2005;23:8520-30.
99. Villeval JL, Cohen-Solal K, Tulliez M, et al. High thrombopoietin production by hematopoietic cells induces a fatal myeloproliferative syndrome in mice. Blood 1997;90:4369-83.
100. Komura E, Tonetti C, Penard-Lacronique V, et al. Role for the nuclear factor kappaB pathway in transforming growth factor-beta1 production in idiopathic myelofibrosis: possible relationship with FK506 binding protein 51 overexpression. Cancer Res 2005;65:3281-9.
101. Vannucchi AM, Bianchi L, Paoletti F, et al. A pathobiologic pathway linking thrombopoietin, GATA-1, and TGF-beta1 in the development of myelofibrosis. Blood 2005;105:3493-501.
102. James C, Ugo V, Casadevall N, Constantinescu SN, Vainchenker WA. JAK2 mutation in myeloproliferative disorders: pathogenesis and therapeutic and scientific prospects. Trends Mol Med 2005;11:46-54.
103. Barbui T. The leukemia controversy in myeloproliferative disorders: is it a natural progression of disease, a secondary sequela of therapy, or a combination of both? Semin Hematol 2004;41:Suppl 3:15-7.
104. Harrison CN, Campbell PJ, Buck G, et al. Hydroxyurea compared with anagrelide in high-risk essential thrombocythemia. N Engl J Med 2005;353:33-45.
105. Cheung B, Radia D, Pantelidis P, Yadegarfar G, Harrison C. The presence of the JAK2 V617F mutation is associated with a higher haemoglobin and increased risk of thrombosis in essential thrombocythaemia. Br J Haematol 2006;132:244-5.
106. Marchioli R, Finazzi G, Landolfi R, et al. Vascular and neoplastic risk in a large cohort of patients with polycythemia vera. J Clin Oncol 2005;23:2224-32.
107. Berk PD, Goldberg JD, Donovan PB, Fruchtman SM, Berlin NI, Wasserman LR. Therapeutic recommendations in polycythemia vera based on Polycythemia Vera Study Group protocols. Semin Hematol 1986;23:132-43.
108. Campbell PJ, Green AR. Management of polycythemia vera and essential thrombocythemia. Hematology Am Soc Hematol Educ Program 2005;201-8.
109. Tefferi A, Murphy S. Current opinion in essential thrombocythemia: pathogenesis, diagnosis, and management. Blood Rev 2001;15:121-31.
110. Campbell PJ, Scott LM, Baxter EJ, Bench AJ, Green AR, Erber WN. Methods for the detection of the JAK2 V617F mutation in human myeloproliferative disorders. Methods Mol Med 2006;125:253-64.
111. Percy MJ, Jones FG, Green AR, Reilly JT, McMullin MF. The incidence of the JAK2 V617F mutation in patients with idiopathic erythrocytosis. Haematologica 2006;91:413-4.
112. James C, Delhommeau F, Marzac C, et al. Detection of JAK2 V617F as a first intention diagnostic test for erythrocytosis. Leukemia 2006;20:350-3.
113. Barosi G, Ambrosetti A, Finelli C, et al. The Italian Consensus Conference on diagnostic criteria for myelofibrosis with myeloid metaplasia. Br J Haematol 1999;104:730-7.
114. McMullin MF, Bareford D, Campbell P, et al. Guidelines for the diagnosis, investigation and management of polycythaemia/erythrocytosis. Br J Haematol 2005;130:174-95.
115. Cortelazzo S, Finazzi G, Ruggeri M, et al. Hydroxyurea for patients with essential thrombocythemia and a high risk of thrombosis. N Engl J Med 1995;332:1132-6.
116. Falanga A, Marchetti M, Vignoli A, Balducci D, Barbui T. Leukocyte-platelet interaction in patients with essential thrombocythemia and polycythemia vera. Exp Hematol 2005;33:523-30.
117. Arellano-Rodrigo E, Alvarez-Larran A, Reverter JC, Villamor N, Colomer D, Cervantes F. Increased platelet and leukocyte activation as contributing mechanisms for thrombosis in essential thrombocythemia and correlation with the JAK2 mutational status. Haematologica 2006;91:169-75.
118. Jones AV, Silver RT, Waghorn K, et al. Minimal molecular response in polycythemia vera patients treated with imatinib or interferon alpha. Blood 2006;107:3339-41.
119. Kiladjian JJ, Cassinat B, Turlure P, et al. High molecular response rate of polycythemia vera patients treated with pegylated interferon alpha-2a. Blood 2006;108:2037-40.