Skip to main content
SpringerLink
Log in

Diagnosis and Treatment of Essential Thrombocythemia and Primary Myelofibrosis

  • Chapter
  • First Online:
Neoplastic Diseases of the Blood

  • 2759 Accesses

Abstract

Essential thrombocythemia (ET) is a clonal stem cell disorder that is characterised by a persistently raised platelet count and an increased risk of venous and arterial thrombosis, as well as haemorrhage. It is an uncommon disorder, with an annual incidence of between 0.6 and 2.5 per 100,000 population, that presents at around 60 years of age, although a second peak occurs at a much younger age, especially in females of child-bearing age. Cases presenting in childhood are uncommon and the rare differential diagnosis of congenital thrombocytosis should be considered.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 269.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Epstein E, Goedel A. Hamorrhagische thrombozythämie bei vascularer schrumpfmilz. Virchows Arch A Pathol Anat Histopathol. 1934;293:233.

    Google Scholar 

  2. Scott LM, Scott MA, Campbell PJ, Green AR. Progenitors homozygous for the V617F mutation occur in most patients with polycythemia vera, but not essential thrombocythemia. Blood. 2006;108:2435–7.

    Article  PubMed  CAS  Google Scholar 

  3. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW. WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon: IARC; 2008.

    Google Scholar 

  4. Campbell PJ, Scott LM, Buck G, Wheatley K, East CL, Marsden JT, Duffy A, Boyd EM, Bench AJ, Scott MA, Vassiliou GS, Milligan DW, Smith SR, Erber WN, Bareford D, Wilkins BS, Reilly JT, Harrison CN, Green AR. Definition of sub-types of essential thrombocythaemia and relation to polycythaemia vera based on JAK2 V617F mutation status: a prospective study. Lancet. 2005;366:1945–53.

    Article  PubMed  CAS  Google Scholar 

  5. Wilkins BS, Erber WN, Bareford D, Buck G, Wheatley K, East CL, Paul B, Harrison CN, Green AR, Campbell PJ. Bone marrow pathology in essential thrombocythemia: interobserver reliability and utility for identifying disease subtypes. Blood. 2008;111:60–70.

    Article  PubMed  CAS  Google Scholar 

  6. Gangat N, Tefferi A, Thanarajasingam G, Patnaik M, Schwager S, Ketterling R, Wolanskyj AP. Cytogenetic abnormalities in essential thrombocythemia: prevalence and prognostic significance. Eur J Haematol. 2009;83:17–21.

    Article  PubMed  CAS  Google Scholar 

  7. Malcovati L, Della Porta MG, Pietra D, Boveri E, Pellagatti A, Gallì A, Travaglino E, Brisci A, Rumi E, Passamonti F, Invernizzi R, Cremonesi L, Boultwood J, Wainscoat JS, Hellström-Lindberg E, Cazzola M. Molecular and clinical features of refractory anemia with ringed sideroblasts associate with marked thrombocytosis. Blood. 2009;114:3538–45.

    Article  PubMed  CAS  Google Scholar 

  8. Cortelazzo S, Finazzi G, Ruggeri M, Vertri O, Galli M, Rodeghiero F, Barbui T. Hydroxyurea for patients with essential thrombocythemia and a high risk of thrombosis. N Eng J Med. 1995;332: 1132–6.

    Article  CAS  Google Scholar 

  9. Harrison CN, Campbell PJ, Buck G, Wheatley K, East CL, Bareford D, Wilkins BS, van der Walt JD, Reilly JT, Grigg AP, Revell P, Woodcock BE, Green AR. Hydroxyurea compared to anagrelide in high-risk essential thrombocythemia. N Engl J Med. 2005;353:33–45.

    Article  PubMed  CAS  Google Scholar 

  10. van Genderen PJJ, Mulder PG, Waleboer M, van de Moesdijk D, Michiels JJ. Prevention and treatment of thrombotic complications in essential thrombocythaemia: efficacy and safety of aspirin. Br J Haematol. 1997;97:179–84.

    Article  PubMed  Google Scholar 

  11. Landolfi R, Marchioli R, Kutti J, Gisslinger H, Tognoni G, Patrono C, Barbui T. Efficacy and safety of low-dose aspirin in polycythemia vera. N Engl J Med. 2004;350:114–24.

    Article  PubMed  CAS  Google Scholar 

  12. Bain BJ. Bone marrow biopsy morbidity: review of 2003. J Clin Pathol. 2005;58:406–8.

    Article  PubMed  CAS  Google Scholar 

  13. Harrison CN. Pregnancy and its management in the Philadelphia negative myeloproliferative diseases. Br J Haematol. 2005;129:293–306.

    Article  PubMed  CAS  Google Scholar 

  14. Gisslinger H, Gotic M, Holowiecki J, Penka M, Thiele J, Kvasnicka HM, Kravovics R, Petrides PE. Final result of the ANAHYDRET-study: non-inferiority of anagrelide compared to hydroxyurea in newly diagnosed WHO-essential thrombocythemia patients. Blood. 2008;112:661a.

    Article  Google Scholar 

  15. Lengfelder E, Griesshammer M, Hehlmann R. Interferon-alpha in the treatment of essential thrombocythemia. Leuk Lymphoma. 1996;22(1):135–42.

    Article  PubMed  Google Scholar 

  16. Kiladjian J-J, Chomienne G, Fenaux P. Interferon-α therapy in bcr-abl-negative myeloproliferative neoplasms. Leukemia. 2008;22:1990–8.

    Article  PubMed  CAS  Google Scholar 

  17. Quintas-Cardama A, Kantarjian H, Manshouri T, Luthra R, Estrov Z, Pierce S, Richie MA, Borthakur G, Konopleva M, Cortes J, Verstovsek S. Pegylated interferon alfa-2a yields high rates of hematologic and molecular response in patients with advanced essential thrombocythemia and polycythemia vera. J Clin Oncol. 2009;10:5418–24.

    Article  Google Scholar 

  18. Finazzi G, Ruggeri M, Rodeghiero F, Barbui T. Second malignancies in patients with essential thrombocythaemia treated with busulphan and hydroxyurea: long term follow up of a randomized clinical trial. Br J Haematol. 2000;110:577–83.

    Article  PubMed  CAS  Google Scholar 

  19. Van de Pette JE, Prochazka AV, Pearson TC, Singh AK, Dickson ER, Wetherley-mein G. Primary essential thrombocythaemia treated with busulphan. Br J Haematol. 1986;62:229–37.

    Article  PubMed  Google Scholar 

  20. Randi ML, Fabris F, Varotto L, Rossi C, Macri C, Girolami A. Haematological complications in polycythaemia vera and thrombocythaemia patients treated with radiophosphorus (32P). Folio Haematol Int Mag Klin Morphol Blutforsch. 1990;117:461–7.

    CAS  Google Scholar 

  21. Skoda R, Prchal JT. Lessons from familial myeloproliferative disorders. Semin Hematol. 2005;42:266–73.

    Article  PubMed  CAS  Google Scholar 

  22. Randi ML, Putti MC. Essemtial thrombocythaemia in children: is a treatment needed? Expert Opin Pharmacother. 2004;5:1009–14.

    Article  PubMed  Google Scholar 

  23. Ruggeri M, Rodeghiero F, Tosetto A, Castaman G, Scognamiglio F, Finazzi G, Delaini F, Mico C, Vannuchi AM, Antonioli E, De Stefano V, Za T, Gugliotta L, Tieghi A, Mazzucconi MG, Santoro C, Barbui T. Postsurgery outcomes in patients with polycythemia vera and essential thrombocythemia: a retrospective survey. Blood. 2008;111:666–71.

    Article  PubMed  CAS  Google Scholar 

  24. Reilly JT. Pathogenesis of idiopathic myelofibrosis: a review. Hematology. 1998;3:205–13.

    Google Scholar 

  25. Reilly JT, Snowden JA, Spearing RL, Fitzgerald PM, Jones N, Watmore A, Potter A. Cytogenetic abnormalities and their prognostic significance in idiopathic myelofibrosis: a study of 106 cases. Br J Haematol. 1997;98:96–102.

    Article  PubMed  CAS  Google Scholar 

  26. Tefferi A, Mesa RA, Schroeder G, Hanson CA, Li CY, Dewald GW. Cytogenetic findings and their clinical relevance in myelofibrosis with myeloid metaplasia. Br J Haematol. 2001;113:763–71.

    Article  PubMed  CAS  Google Scholar 

  27. Dingli D, Grand FH, Mahaffey V, Spurbeck J, Ross FM, Watmore AE, Reilly JT, Cross NC, Dewald GW, Tefferi A. Der(6)t(1;6)(q21-23; p21): a specific cytogenetic abnormality in myelofibrosis with myeloid metaplasia. Br J Haematol. 2005;130:229–32.

    Article  PubMed  CAS  Google Scholar 

  28. Levine RL, Gilliland DG. Myeloproliferative disorders. Blood. 2008;112:2190–8.

    Article  PubMed  CAS  Google Scholar 

  29. Pikman Y, Lee BH, Mercher T, McDowell E, Ebert BL, Gozo M, Cuker A, Wernig G, Moore S, Galinsky I, DeAngelo DJ, Clark JJ, Lee SJ, Golub TR, Wadleigh M, Gilliland DG, Levine RL. MPLW515L is a novel somatic activating mutation in myelofibrosis with myeloid metaplasia. PLoS Med. 2006;3:1140–51.

    Article  CAS  Google Scholar 

  30. Schmitt A, Jouault H, Guichard J, Wendling F, Dronin A, Cramer EM. Pathologic interaction between megakaryocytes and polymorphonuclear leukocytes in myelofibrosis. Blood. 2000;96:1342–7.

    PubMed  CAS  Google Scholar 

  31. Xu M, Bruno E, Chao J, Huang S, Finazzi G, Fruchtman SM, Popat U, Prchal JT, Barosi G, Hoffman R. The constitutive mobilization of CD34+ cells into the peripheral blood in idiopathic myelofibrosis may be due to the action of a number of proteases. Blood. 2005;105:4508–15.

    Article  PubMed  CAS  Google Scholar 

  32. Ciurea SO, Merchant D, Mahmud N, Ishii T, Zhao Y, Hu W, Bruno E, Barosi G, Xu M, Hoffman R. Pivotal contributions of megakaryocytes to the biology of idiopathic myelofibrosis. Blood. 2007;110:986–93.

    Article  PubMed  CAS  Google Scholar 

  33. Cervantes F, Dupriez B, Pereira A, Passamonti F, Reilly JT, Morra E, Vannucchi AM, Mesa RA, Demory JL, Tefferi A. New prognostic scoring system for primary myelofibrosis based on a study of the International Working Group for Myelofibrosis Research and Treatment. Blood. 2009;113:2895–901.

    Article  PubMed  CAS  Google Scholar 

  34. Dupriez B, Morel P, Demory JL, Lai JL, Simon M, Plantier I, Bauters F. Prognostic factors in agnogenic myeloid metaplasia: a report on 195 cases with a new scoring system. Blood. 1996;88:1013–8.

    PubMed  CAS  Google Scholar 

  35. Cervantes F, Barosi G, Demory JL, Reilly J, Guarnone R, Dupriez B, Pereira A, Montserrat E. Myelofibrosis with myeloid metaplasia in young individuals: disease characteristics, prognostic factors and identification of risk groups. Br J Haematol. 1998;102:684–90.

    Article  PubMed  CAS  Google Scholar 

  36. Tam CS, Abruzzo LV, Lin KI, Cortes J, Lynn A, Keating MJ, Thomas DA, Pierce S, Kanttarjian H, Verstovsek S. The role of cytogenetic abnormalities as a prognostic marker in primary myelofibrosis: applicability at the time of diagnosis and later during disease course. Blood. 2009;113:4171–8.

    Article  PubMed  CAS  Google Scholar 

  37. Hidaka T, Shide K, Shimoda H, Kameda T, Toyama K, Katayose K, Kubuki Y, Nagata K, Takenaka K, Akashi K, Okamura T, Niho Y, Mizoguchi H, Omine M, Ozawa K, Harada M, Shimoda K. The impact of cytogenetic abnormalities on the prognosis of primary myelofibrosis: a prospective survey of 202 cases in Japan. Eur J Haematol. 2009;83:328–33.

    Article  PubMed  Google Scholar 

  38. Faoro LN, Tefferi A, Mesa RA. Long-term analysis of the palliative benefit of 2-chlorodeoxyadensoine for myelofibrosis with myeloid metaplasia. Eur J Haematol. 2005;74:117–20.

    Article  PubMed  CAS  Google Scholar 

  39. Cervantes F, Hernández-Boluda JC, Alvarez A, Nadal E, Montserrat E. Danazol treatment of idiopathic myeofibrosis with severe anaemia. Haematologica. 2002;85:595–9.

    Google Scholar 

  40. Hasselbalch HC, Clausen NT, Jensen BA. Successful treatment of anaemia in idiopathic myelofibrosis with recombinant human erythropoietin. Am J Hematol. 2002;70:92–9.

    Article  PubMed  CAS  Google Scholar 

  41. Cervantes F, Alvarez-Larrán A, Hernández-Boluda JC, Sureda A, Torrebadell M, Monserrat E. Erythropoietin treatment of the anaemia of myelofibrosis with myeloid metaplasma: results in 20 patients and review of literatura. Br J Haematol. 2004;127:399–403.

    Article  PubMed  CAS  Google Scholar 

  42. Parmeggiani L, Ferrant A, Rodhain J, Michaux JL, Sokal G. Alpha interferon in the treatment of symptomatic myelofibrosis with myeloid metaplasia. Eur J Haematol. 1987;39:228–32.

    Article  PubMed  CAS  Google Scholar 

  43. Kiladjian JJ, Chomienne C, Fenaux P. Interferon-alpha therapy in bcr-abl-negative myeloproliferative neoplasms. Leukemia. 2008;22:1190–998.

    Article  Google Scholar 

  44. Ianotto JC, Kiladjian JJ, Demory JL, Roy L, Boyer F, Rey J, Dupriez B, Berthou C, Abgrall JF. PEG-IFN-alpha-2a therapy in patients with myelofibrosis: a study of the French Groupe d’Etudes des Myelofibroses (GEM) and France Intergroupe des syndromes Myéloprolifératifs (FIM). Br J Haematol. 2009;146:223–5.

    Article  PubMed  CAS  Google Scholar 

  45. Barosi G, Elliot M, Canepa L, Filippo B, Piccaluga PP, Visani G, Marchetti M, Pozzato G, Zorat F, Tefferi A. Thalidomide in myelofibrosis with myeloid metaplasia: a pooled-analysis of individual pateint data from 5 studies. Leuk Lymphoma. 2002;43:2301–7.

    Article  PubMed  Google Scholar 

  46. Mesa RA, Steensma DP, Pardanani A, Li CY, Elliott M, Kaufmann SH, Wiseman G, Gray LA, Schroeder G, Reeder T, Zeldis JB, Tefferi A. A phase 2 trial of combination low-dose thalidomide and prednisolone for the treatment of myelofibrosis with myeloid metaplasia. Blood. 2003;101:2534–41.

    Article  PubMed  CAS  Google Scholar 

  47. Tefferi A, Lasho TL, Mesa RA, Pardonani A, Ketterling RP, Hanson CA. Lenalidamide therapy in del(5)(q31)-associated myelofibrosis: cytogenetic and JAK2V617F molecular remissions. Leukemia. 2007;21:1827–8.

    Article  PubMed  CAS  Google Scholar 

  48. Tefferi A, Verstovsek S, Barosi G, Passamonti F, Roboz GJ, Gisslinger H, Paquette RL, Cervantes F, Rivera CE, Deeg HJ, Thiele J, Kvasnicka HM, Vardiman JW, Zhang Y, Bekele BN, Mesa RA, Gale RP, Kantarjian HM. Pomalidomide is active in the treatment of anemia asscoaited with myelofibrosis. J Clin Oncol. 2009;27:4563–9.

    Article  PubMed  CAS  Google Scholar 

  49. Steensma DP, Mesa RA, Li CY, Gray L, Tefferi A. Etanercept, a soluble tumor necrosis factor receptor, palliates constitutional symptoms in patients with myelofibsosis with myeloid metaplasia. Blood. 2002;99:2252–4.

    Article  PubMed  CAS  Google Scholar 

  50. Tefferi A, Mesa RA, Gray LA, Steensma DP, Carnoriano JK, Elliott MA, Pardanani A, Ansell SM, Call TG, Colon-Otero G, Schroeder G, Hanson CA, Dewald GW, Kaufmann SH. Phase 2 trial of imatinib mesylate in myelofibrosis with myeloid metaplasia. Blood. 2002;99:3854–6.

    Article  PubMed  CAS  Google Scholar 

  51. Cortes J, Albitar M, Thomas D, Giles F, Furzrock R, Thibault A, Rackoff W, Koller C, O’Brien S, Garcia-Manero G, Talpaz M, Kantarjian H. Efficacy of the farnesyl transferase inhibitor R115777 in chronic myeloid leukaemia and other hematologic malignancies. Blood. 2003;101:1692–7.

    Article  PubMed  CAS  Google Scholar 

  52. Verstovsek S, Kantarjian HM, Mesa RA, Cortes-Franco J, Pardanani AD, Thomas DA, Estrov Z, Bradley EC, Erickson-Viitanen S, Vaddi K, Levy R, Tefferi A. Long-term follow up and optimized dosing of INCB018424 in patients with myelofibrosis: durable clinical, functional and symptomatic response with improved hematological safety. Blood. 2009;114:756a.

    Article  Google Scholar 

  53. Verstovsek S, Odenike O, Scott B, Estrov Z, Cortes J, Thomas DA, Wood J, Ethirajulu K, Lowe A, Zhu HJ, Kantarjian H, Deeg HJ. Phase I dose-escalation trial of SB1518, a novel JAK2/FLT3 ­inhibitor, in acute and chronic myeloid diseases, including primary or post-essential thrombocythemia/polycythemia vera myelofibrosis. Blood. 2009;114:3905a.

    Google Scholar 

  54. Pardanani AD, Gotlib JR, Jamieson C, Cortes J, Talpaz M, Stone R, Silverman MH, Shorr J, Gilliland DG, Tefferi A. A phase I evaluation of TG101348, a selective inhibitor, in myelofibrosis: clinical response is accompanied by significant reduction in JAK2V617F allele burden. Blood. 2009;114:755a.

    Google Scholar 

  55. Mesa RA, Nagorney DS, Schwager S, Alfred J, Tefferi A. Palliative goals, patient selection, and perioperative platelet management: outcomes and lessons from 3 decades of splenectomy for myelofibrosis with myeloid metaplasia at the Mayo Clinic. Cancer. 2006;167:361–70.

    Article  Google Scholar 

  56. Bouabdallah R, Coso D, Gonzague-Casabianca L, Alzieu C, Resbeut M, Gastaut JA. Safety and efficacy of splenic irradiation in the treatment of patients with idiopathic myelofibrosis: a report on 15 patients. Leuk Res. 2000;24:491–5.

    Article  PubMed  CAS  Google Scholar 

  57. Guardiola P, Anderson JE, Gluckman E. Myelofibrosis with myeloid metaplasia. N Engl J Med. 2000;343:659.

    Article  PubMed  CAS  Google Scholar 

  58. Kerbauy DM, Gooley TA, Sale GE, Flowers ME, Doney KC, Georges GE, Greene JE, Linenberger M, Petersdorf E, Sansmaier BM, Scott BL, Sorror M, Stirewalt DL, Stewart FM, Witherspoon RP, Storb R, Appelbaum FR, Deeg HJ. Hematopoietic cell transplantation as curative therapy therapy for idiopathic myelofibrosis, advanced polycythemia vera, and essential thrombocythemia. Biol Blood Marrow Transplant. 2007;13:355–65.

    Article  PubMed  Google Scholar 

  59. Deeg HJ, Gooley TA, Flowers ME, Sale GE, Slattery JT, Anasetti C, Chauncey TR, Doney K, Georges GE, Kiem HP, Martin PJ, Petersdorf EW, Radich J, Sanders JE, Sandmaier BM, Warren EH, Witherspoon RP, Storb R, Appelbaum FR. Allogeneic hematopoietic stem cell transplantation for myelofibrosis. Blood. 2003;102:3912–8.

    Article  PubMed  CAS  Google Scholar 

  60. Guardiola P, Anderson JE, Bandini G, Cervantes F, Runde V, Arcese W, Bacigalupo A, Przepiorka D, O’Donnell MR, Polchi P, Buzyn A, Sutton L, Cazals-Hatem D, Sale G, de Witte T, Deeg HJ, Gluckman E. Allogeneic stem cell transplantation for agnogenic myeloid metaplasia: a European Group for Blood and Marrow Transplantation. Blood. 1999;93:2831–8.

    PubMed  CAS  Google Scholar 

  61. Ciurea SO, Sadegi B, Wilbur A, Alaiozian-Angelova V, Gaitonde S, Dobogai LC, Akard LP, Hoffman R, Rondelli D. Effects of extensive splenomegaly in patients with myelofibrosis undergoing a reduced intensity allogeneic stem cell transplantion. Br J Haematol. 2008;141:80–3.

    Article  PubMed  Google Scholar 

  62. Byrne JL, Beshti H, Clark D, Ellis I, Haynes AP, Das-Gupta E, Russell NH. Induction of remission after donor leucocyte infusion for the treatment of relapsed chronic idiopathic myelofibrosis ­following allogeneic transplantation: evidence for a ‘graft vs myelofibrosis’ effect. Br J Haematol. 1999;108:430–3.

    Article  Google Scholar 

  63. Cervantes F, Rovira M, Urbano-Ispizua A, Rozman M, Carreras E, Montserrat E. Complete remission of idiopathic myelofibrosis ­following donor lymphocyte infusion after failure of allogeneic transplantation: demonstration of a graft-versus-myelofibrosis effect. Bone Marrow Transplant. 2000;26:697–9.

    Article  PubMed  CAS  Google Scholar 

  64. Hessling J, Kroger N, Werner M, Zabelina T, Hansen A, Kordes U, Ayuk FA, Renges H, Panse J, Erttmann R, Zander AR. Dose-reduced conditioning regimen followed by allogeneic stem cell transplantation in patients with myelofibrosis with myeloid metaplasia. Br J Haematol. 2002;119:769–72.

    Article  PubMed  Google Scholar 

  65. Devine SM, Hoffman R, Verma A, Shah R, Bradlow BA, Stock W, Maynard V, Jessop E, Peace D, Humi M, Thomason D, Chen YH, van Besien K. Allogeneic blood cell transplantation following reduced-intensity conditioning is effective therapy for older patients with myelofibrosis with myeloid metaplasia. Blood. 2002;99:2255–8.

    Article  PubMed  CAS  Google Scholar 

  66. Kröger N, Zabelina T, Scheider H, Panse J, Ayuk F, Stute N, Fehse N, Waschke O, Fehse B, Kvasnicka HM, Thiele J, Zander A. Pilot study of reduced-intensity conditioning followed by allogeneic stem cell transplantation from related and unrelated donors in patients with myelofibrosis. Br J Haematol. 2005;128:690–7.

    Article  PubMed  Google Scholar 

  67. Kröger N, Holler E, Krobbe G, Bornhäuser M, Schwerdfeger R, Baumann H, Ngaler A, Bethge W, Stelljes M, Uharek L, Wandt H, Burchert A, Corradini P, Schubert J, Kaufmann M, Dreger P, Wulf GG, Einsele H, Zabelina T, Kvasnicka HM, Thiele J, Brand R, Zander AR, Niederwiesser D, de Witte TM. Allogeneic stem cell transplantation after reduced-intensity conditioning in patients with myelofibrosis: a prospective, multicenter study of the Chronic Leukemia Working Party of the European Group for Blood and Marrow Transplantation. Blood. 2009;114:5264–70.

    Article  PubMed  Google Scholar 

  68. Anderson JE, Tefferi A, Craig F, Holmberg L, Chauncey T, Appelbaum FR, Guardiola P, Callander N, Freytes C, Gazitt Y, Razvillas B, Deeg HJ. Myeloablation and autologous peripheral blood stem cell rescue results in hematologic and clinical responses in patients with myelofibrosis. Blood. 2001;98:586–93.

    Article  PubMed  CAS  Google Scholar 

  69. Barosi G, Besses C, Birgegard G, Briere J, Cervantes F, Finazzi G, Gisslinger H, Griesshammer M, Gugliotta L, Harrison C, Hasselblach H, Lengfelder E, Reilly JT, Michiels JJ, Barbui T. A unified definition of clinical resistance/intolerance to hydroxyurea in essential thrombocythemia: results of a consensus process by an international working group. Leukemia. 2007;21:277–80.

    Article  PubMed  CAS  Google Scholar 

Download references

Acknowledgements

The author is grateful to both Professor T.C. Pearson and Dr. Maria Messinezy for contributions to previous versions of this chapter.

Author information

Authors and Affiliations

  1. Department of Hematology, Central Sheffield University Hospital, Glossop Road, Sheffield, South Yorkshire, S10 2JF, UK

    John T. Reilly B.Sc., M.D., F.R.C.P., F.R.C.Path.

Authors
  1. John T. Reilly B.Sc., M.D., F.R.C.P., F.R.C.Path.
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to John T. Reilly B.Sc., M.D., F.R.C.P., F.R.C.Path. .

Editor information

Editors and Affiliations

  1. Beth Israel Hospital, Cancer Center, St. Lukes-Roosevelt Hospital Center, 10th Avenue 1000, New York, 10019, New York, USA

    Peter H. Wiernik

  2. , Department of Hematology, Imperial College of London, Du Cane Road 150, London, W12 0NN, United Kingdom

    John M. Goldman

  3. Continuum Cancer Centers, Department of Medicine, St. Luke's-Roosevelt Hospital Center, 10th Avenue 1000, New York, 10019, New York, USA

    Janice P. Dutcher

  4. , Division of Hematology, Mayo Clinic, First Street SW. 200, Rochester, 55905, Minnesota, USA

    Robert A. Kyle

Rights and permissions

Reprints and permissions

Copyright information

© 2013 Springer Science+Business Media New York

About this chapter

Cite this chapter

Reilly, J.T. (2013). Diagnosis and Treatment of Essential Thrombocythemia and Primary Myelofibrosis. In: Wiernik, P., Goldman, J., Dutcher, J., Kyle, R. (eds) Neoplastic Diseases of the Blood. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-3764-2_12

Download citation

  • DOI: https://doi.org/10.1007/978-1-4614-3764-2_12

  • Published:

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4614-3763-5

  • Online ISBN: 978-1-4614-3764-2

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation