Seminars in Hematology
Volume 42, Issue 4 , Pages 206-220 , October 2005

Polycythemia Vera: Scientific Advances and Current Practice

  • Ayalew Tefferi

      Affiliations

    • Mayo Clinic College of Medicine, Rochester, MN.
    • Corresponding Author InformationAddress correspondence to Ayalew Tefferi, MD, Division of Hematology, Mayo Clinic, 200 First St SW, Rochester, MN 55905.
    • ,
  • Jerry L. Spivak

      Affiliations

    • Johns Hopkins University School of Medicine, Baltimore, MD.

References 

  1. Vaquez MH . Sur une forme speciale de cyanose s’accompagnant d’hyperglobulie excessive et persistante . CR Soc Biol (Paris) . 1892;44:384–388
  2. Osler W . Chronic cyanosis, with polycythemia and enlarged spleen (A new clinical entity) . Am J Med Sci . 1903;126:187–201
  3. Turk W . Beitrage zur kenntnis des symptomenbildes polyzythamie mit milztumor und zyanose . Wien Med Wochenschr . 1904;17:153–160 189-193
  4. Low J , Popper H . Wien Med Wochenschr . 1910;21:357
  5. Lesourd L , Pagniez P . Comptes Rendus Seances Soc Biol . 1910;68:746
  6. Brown GE , Giffin HZ . Peripheral arterial disease in polycythemia vera . Arch Intern Med . 1930;46:705–717
  7. Stephens DJ , Kaltreider NL . The therapeutic use of venesection in polycythemia . Ann Intern Med . 1937;10:1565
  8. Rosenthal N , Bassen FA . Course of polycythemia . Arch Intern Med . 1938;62:903–917
  9. Tinney WS , Hall BE , Giffen HZ . Hematologic complications of P. vera . Proc Staff Meet Mayo Clin . 1943;18:227–230
  10. Videbaek A . Polycythemia vera. Course and prognosis . Acta Med Scand . 1950;138:179
  11. Hirsch R . Generalized osteosclerosis with chronic polycythemia vera . Arch Pathol . 1935;19:91–97
  12. Dameshek W . Some speculations on the myeloproliferative syndromes . Blood . 1951;6:372–375
  13. Fialkow PJ , Gartler SM , Yoshida A . Clonal origin of chronic myelocytic leukemia in man . Proc Natl Acad Sci U S A . 1967;58:1468–1471
  14. 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;58:916–919
  15. 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;295:913–916
  16. Jacobson RJ , Salo A , Fialkow PJ . Agnogenic myeloid metaplasia (A clonal proliferation of hematopoietic stem cells with secondary myelofibrosis) . Blood . 1978;51:189–194
  17. Tefferi A , Thibodeau SN , Solberg LA . Clonal studies in the myelodysplastic syndrome using X-linked restriction fragment length polymorphisms . Blood . 1990;75:1770–1773
  18. Tefferi A , Lasho TL , Brockman SR , Elliott MA , Dispenzieri A , Pardanani A . FIP1L1-PDGFRA and c-kit D816V mutation-based clonality studies in systemic mast cell disease associated with eosinophilia . Haematologica . 2004;89:871–873
  19. Tefferi A . Chronic myeloid disorders (Classification and treatment overview) . Semin Hematol . 2001;38:1–4
  20. Tefferi A, Gilliland DG: Classification of myeloproliferative disorders: From Dameshek towards a semi-molecular system. Best Pract Res Clin Haematol (in press)
  21. Tefferi A , Gilliland DG . The JAK2 V617F tyrosine kinase mutation in myeloproliferative disorders (Status report and immediate implications for disease classification and diagnosis) . Mayo Clin Proc . 2005;80:947–958
  22. Kralovics R , Stockton DW , Prchal JT . Clonal hematopoiesis in familial polycythemia vera suggests the involvement of multiple mutational events in the early pathogenesis of the disease . Blood . 2003;102:3793–3796
  23. Finazzi G , Caruso V , Marchioli R , et al.   Acute leukemia in polycythemia vera. An analysis of 1,638 patients enrolled in a prospective observational study . Blood . 2004;105:2664–2670
  24. Danish EH , Rasch CA , Harris JW . Polycythemia vera in childhood (Case report and review of the literature) . Am J Hematol . 1980;9:421–428
  25. Poggi V , Migliorati R , Fiore M , Amoroso R , Ghio R , Fiorillo A . Polycythemia vera (A new case report with onset in infancy) . Haematologica . 1984;69:458–463
  26. Heilmann E , Klein CE , Beck JD . Primary polycythaemia in childhood and adolescence . Folia Haematol Int Mag Klin Morphol Blutforsch . 1983;110:935–941
  27. Najean Y , Rain JD , Billotey C . Epidemiological data in polycythaemia vera (A study of 842 cases) . Hematol Cell Ther . 1998;40:159–165
  28. Passamonti F , Rumi E , Pungolino E , et al.   Life expectancy and prognostic factors for survival in patients with polycythemia vera and essential thrombocythemia . Am J Med . 2004;117:755–761
  29. Dameshek W . Physiopathology and course of polycythemia vera as related to therapy . JAMA . 1950;142:790–797
  30. Elliott MA , Tefferi A . Thrombosis and haemorrhage in polycythaemia vera and essential thrombocythaemia . Br J Haematol . 2005;128:275–290
  31. Finazzi G . A prospective analysis of thrombotic events in the European collaboration study on low-dose aspirin in polycythemia (ECLAP) . Pathol Biol (Paris) . 2004;52:285–288
  32. Diehn F , Tefferi A . Pruritus in polycythaemia vera (Prevalence, laboratory correlates and management) . Br J Haematol . 2001;115:619–621
  33. Michiels JJ , Berneman Z , Schroyens W , van Urk H . Aspirin-responsive painful red, blue, black toe, or finger syndrome in polycythemia vera associated with thrombocythemia . Ann Hematol . 2003;82:153–159
  34. Najfeld V , Montella L , Scalise A , Fruchtman S . Exploring polycythaemia vera with fluorescence in situ hybridization (Additional cryptic 9p is the most frequent abnormality detected) . Br J Haematol . 2002;119:558–566
  35. Zamora L , Espinet B , Florensa L , et al.   Is fluorescence in situ hybridization a useful method in diagnosis of polycythemia vera patients? . Cancer Genet Cytogenet . 2004;151:139–145
  36. Gribble SM , Reid AG , Bench AJ , et al.   Molecular cytogenetics of polycythaemia vera (Lack of occult rearrangements detectable by 20q LSP screening, CGH, and M-FISH) . Leukemia . 2003;17:1419–1421
  37. Chen Z , Notohamiprodjo M , Guan XY , et al.   Gain of 9p in the pathogenesis of polycythemia vera . Genes Chromosomes Cancer . 1998;22:321–324
  38. Westwood NB , Gruszka-Westwood AM , Atkinson S , Pearson TC . Polycythemia vera (analysis of DNA from blood granulocytes using comparative genomic hybridization) . Haematologica . 2001;86:464–469
  39. Kralovics R , Guan Y , Prchal JT . Acquired uniparental disomy of chromosome 9p is a frequent stem cell defect in polycythemia vera . Exp Hematol . 2002;30:229–236
  40. Kralovics R , Buser AS , Teo SS , et al.   Comparison of molecular markers in a cohort of patients with chronic myeloproliferative disorders . Blood . 2003;102:1869–1871
  41. Baxter EJ , Scott LM , Campbell PJ , et al.   Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders . Lancet . 2005;365:1054–1061
  42. James C , Ugo V , Le Couedic JP , et al.   A unique clonal JAK2 mutation leading to constitutive signalling causes polycythaemia vera . Nature . 2005;434:1144–1148
  43. Kralovics R , Passamonti F , Buser AS , et al.   A gain of function mutation in Jak2 is frequently found in patients with myeloproliferative disorders . N Engl J Med . 2005;352:1779–1790
  44. 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;7:387–397
  45. 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 the myelodysplastic syndrome . Blood . 2005;106:1207–1209
  46. Jones AV , Kreil S , Zoi K , et al.   Widespread occurrence of the JAK2 V617F mutation in chronic myeloproliferative disorders . Blood . 2005; prepublished online May 26,
  47. Prchal JF , Axelrad AA . Bone-marrow responses in polycythemia vera . N Engl J Med . 1974;290:1382; (letter)
  48. Fisher MJ , Prchal JF , Prchal JT , D’Andrea D . Anti-erythropoietin (EPO) receptor monoclonal antibodies distinguish EPO-dependent and EPO-independent erythroid progenitors in polycythemia vera . Blood . 1994;84:1982–1991
  49. Eridani S , Dudley JM , Sawyer BM , Pearson TC . Erythropoietic colonies in a serum-free system (Results in primary proliferative polycythaemia and thrombocythaemia) . Br J Haematol . 1987;67:387–391
  50. Dai CH , Krantz SB , Green WF , Gilbert HS . Polycythaemia vera. III. Burst-forming units-erythroid (BFU-E) response to stem cell factor and c-kit receptor expression . Br J Haematol . 1994;86:12–21
  51. Dai CH , Krantz SB , Means RT , Horn ST , Gilbert HS . Polycythemia vera blood burst-forming units-erythroid are hypersensitive to interleukin-3 . J Clin Invest . 1991;87:391–396
  52. Correa PN , Eskinazi D , Axelrad AA . Circulating erythroid progenitors in polycythemia vera are hypersensitive to insulin-like growth factor-1 in vitro (Studies in an improved serum-free medium) . Blood . 1994;83:99–112
  53. 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;338:564–571
  54. Mirza AM , Correa PN , Axelrad AA . Increased basal and induced tyrosine phosphorylation of the insulin-like growth factor I receptor beta subunit in circulating mononuclear cells of patients with polycythemia vera . Blood . 1995;86:877–882
  55. Casadevall N , Vainchenker W , Lacombe C , et al.   Erythroid progenitors in polycythemia vera (Demonstration of their hypersensitivity to erythropoietin using serum free cultures) . Blood . 1982;59:447–451
  56. Eaves CJ , Eaves AC . Erythropoietin (Ep) dose-response curves for three classes of erythroid progenitors in normal human marrow and in patients with polycythemia vera . Blood . 1978;52:1196–1210
  57. Cashman J , Henkelman D , Humphries K , Eaves C , Eaves A . Individual BFU-E in polycythemia vera produce both erythropoietin dependent and independent progeny . Blood . 1983;61:876–884
  58. 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;32:179–187
  59. Zwicky C , Theiler L , Zbaren K , Ischi E , Tobler A . The predictive value of clonogenic stem cell assays for the diagnosis of polycythaemia vera . Br J Haematol . 2002;117:598–604
  60. Dobo I , Donnard M , Girodon F , et al.   Standardization and comparison of endogenous erythroid colony assays performed with bone marrow or blood progenitors for the diagnosis of polycythemia vera . Hematol J . 2004;5:161–167
  61. Juvonen E , Partanen S , Ikkala E , Ruutu T . Megakaryocytic colony formation in polycythaemia vera and secondary erythrocytosis . Br J Haematol . 1988;69:441–444
  62. Juvonen E , Partanen S , Ruutu T . Colony formation by megakaryocytic progenitors in essential thrombocythaemia . Br J Haematol . 1987;66:161–164
  63. Battegay EJ , Thomssen C , Nissen C , Gudat F , Speck B . Endogenous megakaryocyte colonies from peripheral blood in precursor cell cultures of patients with myeloproliferative disorders . Eur J Haematol . 1989;42:321–326
  64. Florensa L , Besses C , Woessner S , et al.   Endogenous megakaryocyte and erythroid colony formation from blood in essential thrombocythaemia . Leukemia . 1995;9:271–273
  65. Axelrad AA , Eskinazi D , Correa PN , Amato D . Hypersensitivity of circulating progenitor cells to megakaryocyte growth and development factor (PEG-rHu MGDF) in essential thrombocythemia . Blood . 2000;96:3310–3321
  66. Kobayashi S , Teramura M , Hoshino S , Motoji T , Oshimi K , Mizoguchi H . Circulating megakaryocyte progenitors in myeloproliferative disorders are hypersensitive to interleukin-3 . Br J Haematol . 1993;83:539–544
  67. Montagna C , Massaro P , Morali F , Foa P , Maiolo AT , Eridani S . In vitro sensitivity of human erythroid progenitors to hemopoietic growth factors (Studies on primary and secondary polycythemia) . Haematologica . 1994;79:311–318
  68. Dai CH , Krantz SB , Dessypris EN , Means RT , Horn ST , Gilbert HS . Polycythemia vera. II. Hypersensitivity of bone marrow erythroid, granulocyte-macrophage, and megakaryocyte progenitor cells to interleukin-3 and granulocyte-macrophage colony-stimulating factor . Blood . 1992;80:891–899
  69. Martin JM , Gandhi K , Jackson WR , Dessypris EN . Hypersensitivity of polycythemia vera megakaryocytic progenitors to thrombopoietin . Blood . 1996;88:363a; (abstr)
  70. Michl P , Spoettl G , Engelhardt D , Weber MM . Alterations of the insulin-like growth factor system in patients with polycythemia vera . Mol Cell Endocrinol . 2001;181:189–197
  71. Dai CH , Krantz SB , Sawyer ST . Polycythemia vera .5. Enhanced proliferation and phosphorylation due to vanadate are diminished in polycythemia vera erythroid progenitor cells—A possible defect of phosphatase activity in polycythemia vera . Blood . 1997;89:3574–3581
  72. Dai C , Chung IJ , Krantz SB . Increased erythropoiesis in polycythemia vera is associated with increased erythroid progenitor proliferation and increased phosphorylation of Akt/PKB . Exp Hematol . 2005;33:152–158
  73. Means RT , Krantz SB , Sawyer ST , Gilbert HS . Erythropoietin receptors in polycythemia vera . J Clin Invest . 1989;84:1340–1344
  74. Hess G , Rose P , Gamm H , Papadileris S , Huber C , Seliger B . Molecular analysis of the erythropoietin receptor system in patients with polycythaemia vera . Br J Haematol . 1994;88:794–802
  75. Mittelman M , Gardyn J , Carmel M , Malovani H , Barak Y , Nir U . Analysis of the erythropoietin receptor gene in patients with myeloproliferative and myelodysplastic syndromes . Leuk Res . 1996;20:459–466
  76. Le Couedic JP , Mitjavila MT , Villeval JL , et al.   Missense mutation of the erythropoietin receptor is a rare event in human erythroid malignancies . Blood . 1996;87:1502–1511
  77. Emanuel PD , Eaves CJ , Broudy VC , et al.   Familial and congenital polycythemia in three unrelated families . Blood . 1992;79:3019–3030
  78. Li J , Bench AJ , Huntly BJ , Green AR . Mutation and methylation analysis of the transforming growth factor beta receptor II gene in polycythaemia vera . Br J Haematol . 2001;115:872–880
  79. Moliterno AR , Hankins WD , Spivak JL . Impaired expression of the thrombopoietin receptor by platelets from patients with polycythemia vera . N Engl J Med . 1998;338:572–580
  80. Moliterno AR , Spivak JL . Posttranslational processing of the thrombopoietin receptor is impaired in polycythemia vera . Blood . 1999;94:2555–2561
  81. Bock O , Schlue J , Mengel M , Busche G , Serinsoz E , Kreipe H . Thrombopoietin receptor (Mpl) expression by megakaryocytes in myeloproliferative disorders . J Pathol . 2004;203:609–615
  82. Yoon SY , Li CY , Tefferi A . Megakaryocyte c-Mpl expression in chronic myeloproliferative disorders and the myelodysplastic syndrome (Immunoperoxidase staining patterns and clinical correlates) . Eur J Haematol . 2000;65:170–174
  83. Duensing S , Duensing A , Meran JG , et al.   Molecular detection of c-mpl thrombopoietin receptor gene expression in chronic myeloproliferative disorders . Mol Pathol . 1999;52:146–150
  84. Klippel S , Strunck E , Temerinac S , et al.   Quantification of PRV-1 mRNA distinguishes Polycythemia vera from secondary erythrocytosis . Blood . 2003;102:3569–3574
  85. Moliterno AR , Williams DM , Gutierrez-Alamillo LI , Salvatori R , Ingersoll RG , Spivak JL . Mpl Baltimore (A thrombopoietin receptor polymorphism associated with thrombocytosis) . Proc Natl Acad Sci U S A . 2004;101:11444–11447
  86. Stroncek DF , Caruccio L , Bettinotti M . CD177 (A member of the Ly-6 gene superfamily involved with neutrophil proliferation and polycythemia vera) . J Transl Med . 2004;2:8
  87. Passamonti F , Pietra D , Malabarba L , et al.   Clinical significance of neutrophil CD177 mRNA expression in Ph-negative chronic myeloproliferative disorders . Br J Haematol . 2004;126:650–656
  88. Pellagatti A , Vetrie D , Langford CF , et al.   Gene expression profiling in polycythemia vera using cDNA microarray technology . Cancer Res . 2003;63:3940–3944
  89. Sirhan S , Lasho TL , Elliott MA , Tefferi A . Neutrophil polycythemia rubra vera-1 expression in classic and atypical myeloproliferative disorders and laboratory correlates . Haematologica . 2005;90:406–408
  90. Bock O , Serinsoz E , Neusch M , Schlue J , Kreipe H . The polycythaemia rubra vera-1 gene is constitutively expressed by bone marrow cells and does not discriminate polycythaemia vera from reactive and other chronic myeloproliferative disorders . Br J Haematol . 2003;123:472–474
  91. Passamonti F , Pietra D , Rumi E , et al.   PRV-1 and its correlation with treatments and disease status in 210 patients with polycythemia vera and essential thrombocythemia . Leukemia . 2005;19:888–889
  92. Liu E , Jelinek J , Pastore YD , Guan Y , Prchal JF , Prchal JT . Discrimination of polycythemias and thrombocytoses by novel, simple, accurate clonality assays and comparison with PRV-1 expression and BFU-E response to erythropoietin . Blood . 2003;101:3294–3301
  93. 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
  94. Dai C , Krantz SB . Increased expression of the INK4a/ARF locus in polycythemia vera . Blood . 2001;97:3424–3432
  95. Brockman JL , Schroeder MD , Schuler LA . PRL activates the cyclin D1 promoter via the Jak2/Stat pathway . Mol Endocrinol . 2002;16:774–784
  96. 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;98:181–191
  97. Dolznig H , Habermann B , Stangl K , et al.   Apoptosis protection by the Epo target Bcl-X(L) allows factor-independent differentiation of primary erythroblasts . Curr Biol . 2002;12:1076–1085
  98. Tong W , Zhang J , Lodish H . Lnk inhibits erythropoiesis and Epo-dependent JAK2 activation and downstream signaling pathways . Blood . 2005;105:4604–4612
  99. Asimakopoulos FA , Hinshelwood S , Gilbert JGR , et al.   The gene encoding hematopoietic cell phosphatase (Shp-1) is structurally and transcriptionally intact in polycythemia vera . Oncogene . 1997;14:1215–1222
  100. Sui XW , Krantz SB , Zhao ZH . Identification of increased protein tyrosine phosphatase activity in polycythemia vera erythroid progenitor cells . Blood . 1997;90:651–657
  101. Xu MJ , Sui X , Zhao R , Dai C , Krantz SB , Zhao ZJ . PTP-MEG2 is activated in polycythemia vera erythroid progenitor cells and is required for growth and expansion of erythroid cells . Blood . 2003;102:4354–4360
  102. Najean Y , Mugnier P , Dresch C , Rain JD . Polycythaemia vera in young people (An analysis of 58 cases diagnosed before 40 years) . Br J Haematol . 1987;67:285–291
  103. Jantunen R , Juvonen E , Ikkala E , Oksanen K , Anttila P , Ruutu T . Development of erythrocytosis in the course of essential thrombocythemia . Ann Hematol . 1999;78:219–222
  104. Barosi G , Baraldi A , Cazzola M , et al.   Polycythaemia following splenectomy in myelofibrosis with myeloid metaplasia. A reorganization of erythropoiesis . Scand J Haematol . 1984;32:12–18
  105. Perez-Encinas M , Bello JL , Perez-Crespo S , De Miguel R , Tome S . Familial myeloproliferative syndrome . Am J Hematol . 1994;46:225–229
  106. 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;23:132–143
  107. Spivak JL . Polycythemia vera (Myths, mechanisms, and management) . Blood . 2002;100:4272–4290
  108. Wasserman LR . Polycythemia vera (Its course and treatment; relation to myeloid metaplasia and leukemia) . Bull N Y Acad Med . 1954;30:343–375
  109. Spivak JL , Jie C , Williams DM , Moliterno AR . Gender counts (Defining the core genetic profile of polycythemia) . Blood . 2004;104:190a; (abstr)
  110. Pearson TC , Wetherley-Mein G . Vascular occlusive episodes and venous haematocrit in primary proliferative polycythaemia . Lancet . 1978;2:1219–1222
  111. Bachleitner-Hofmann T , Grumbeck E , Gisslinger H . Oral anticoagulants as secondary prophylaxis of thrombosis in patients with polycythemia vera (A retrospective analysis of 15 patients) . Thromb Res . 2003;112:229–232
  112. Lamy T , Devillers A , Bernard M , et al.   Inapparent polycythemia vera—An unrecognized diagnosis . Am J Med . 1997;102:14–20
  113. Berk PD , Goldberg JD , Silverstein MN , et al.   Increased incidence of acute leukemia in polycythemia vera associated with chlorambucil therapy . N Engl J Med . 1981;304:441–447
  114. Dingli D , Utz JP , Krowka MJ , Oberg AL , Tefferi A . Unexplained pulmonary hypertension in chronic myeloproliferative disorders . Chest . 2001;120:801–808
  115. Michiels JJ . Erythromelalgia and vascular complications in polycythemia vera . Semin Thromb Hemost . 1997;23:441–454
  116. Budde U , Scharf RE , Franke P , Hartmann-Budde K , Dent J , Ruggeri ZM . Elevated platelet count as a cause of abnormal von Willebrand factor multimer distribution in plasma . Blood . 1993;82:1749–1757
  117. Wasserman LR . The treatment of polycythemia. A panel discussion . Blood . 1968;32:483–487
  118. Pearson TC , Messinezy M . The diagnostic criteria of polycythaemia rubra vera . Leuk Lymphoma . 1996;22:87–93
  119. Michiels JJ , Juvonen E . Proposal for revised diagnostic criteria of essential thrombocythemia and polycythemia vera by the Thrombocythemia Vera Study Group . Semin Thromb Hemost . 1997;23:339–347
  120. Murphy S . Diagnostic criteria and prognosis in polycythemia vera and essential thrombocythemia . Semin Hematol . 1999;36:9–13
  121. Pearson TC . Evaluation of diagnostic criteria in polycythemia vera . Semin Hematol . 2001;38:21–24
  122. Pierre R , Imbert M , Thiele J , Vardiman JW , Brunning RD , Flandrin G . Polycythemia vera . In:  Jaffe ES ,  Harris NL ,  Stein H ,  Vardiman JW editor. World health organization (WHO) Classification of Tumors (Pathology and Genetics of Tumors of Haematopoietic and Lymphoid Tissues) . Lyon, France: IARC Press; 2001;p. 32–34
  123. Thiele J , Kvasnicka HM , Muehlhausen K , Walter S , Zankovich R , Diehl V . Polycythemia rubra vera versus secondary polycythemias. A clinicopathological evaluation of distinctive features in 199 patients . Pathol Res Pract . 2001;197:77–84
  124. Wright RR , Tono M , Pollycove M . Blood volume . Semin Nucl Med . 1975;5:63–78
  125. Pearson TC , Guthrie DL , Simpson J , et al.   Interpretation of measured red cell mass and plasma volume in adults (Expert Panel on Radionuclides of the International Council for Standardization in Haematology) . Br J Haematol . 1995;89:748–756
  126. Panel on Diagnostic Applications of Radioisotopes in Haematology . Standard techniques for the measurement of red-cell and plasma volume. A report by the International Committee for Standardization in Hematology (ICSH) . Br J Haematol . 1973;25:801–814
  127. Balga I , Solenthaler M , Furlan M . Should whole-body red cell mass be measured or calculated? . Blood Cells Mol Dis . 2000;26:25–31
  128. Fairbanks VF . Commentary (Should whole-body red cell mass be measured or calculated) . Blood Cells Mol Dis . 2000;26:32–36
  129. Hlad CJ , Tanz R . An analysis of technical errors in radioalbumin blood volume methods and presentation of a modified method . J Lab Clin Med . 1958;52:289–298
  130. Hinghofer-Szalkay H , Greenleaf JE . Continuous monitoring of blood volume changes in humans . J Appl Physiol . 1987;63:1003–1007
  131. Leslie WD , Dupont JO , Peterdy AE . Effect of obesity on red cell mass results . J Nucl Med . 1999;40:422–428
  132. Sirhan S , Fairbanks VF , Tefferi A . Red cell mass and plasma volume measurements in polycythemia . Cancer . 2005;104:213–215
  133. Messinezy M , Westwood NB , El-Hemaidi I , Marsden JT , Sherwood RS , Pearson TC . Serum erythropoietin values in erythrocytoses and in primary thrombocythaemia . Br J Haematol . 2002;117:47–53
  134. Mossuz P , Girodon F , Donnard M , et al.   Diagnostic value of serum erythropoietin level in patients with absolute erythrocytosis . Haematologica . 2004;89:1194–1198
  135. Diez-Martin JL , Graham DL , Petitt RM , Dewald GW . Chromosome studies in 104 patients with polycythemia vera . Mayo Clin Proc . 1991;66:287–299
  136. Tefferi A . Polycythemia vera (A comprehensive review and clinical recommendations) . Mayo Clin Proc . 2003;78:174–194
  137. Temerinac S , Klippel S , Strunck E , et al.   Cloning of PRV-1, a novel member of the uPAR receptor superfamily, which is overexpressed in polycythemia rubra vera . Blood . 2000;95:2569–2576
  138. Koch CA , Lasho TL , Tefferi A . Platelet-rich plasma serotonin levels in chronic myeloproliferative disorders (Evaluation of diagnostic use and comparison with the neutrophil PRV-1 assay) . Br J Haematol . 2004;127:34–39
  139. Alcocer L . Statins for everybody? New evidence on the efficacy and safety of the inhibitors of HMG Co-A reductase . Am J Ther . 2003;10:423–428
  140. Teofili L , Martini M , Luongo M , et al.   Overexpression of the polycythemia rubra vera-1 gene in essential thrombocythemia . J Clin Oncol . 2002;20:4249–4254
  141. Florensa L , Besses C , Zamora L , et al.   Endogenous erythroid and megakaryocytic circulating progenitors, HUMARA clonality assay, and PRV-1 expression are useful tools for diagnosis of polycythemia vera and essential thrombocythemia . Blood . 2004;103:2427–2428
  142. Reid CD . The significance of endogenous erythroid colonies (EEC) in haematological disorders . Blood Rev . 1987;1:133–140
  143. Horikawa Y , Matsumura I , Hashimoto K , et al.   Markedly reduced expression of platelet C-Mpl receptor in essential thrombocythemia . Blood . 1997;90:4031–4038
  144. Chauveau J , Fert V , Morel AM , Delaage MA . Rapid and specific enzyme immunoassay of serotonin . Clin Chem . 1991;37:1178–1184
  145. Maurer-Spurej E , Dyker K , Gahl WA , Devine DV . A novel immunocytochemical assay for the detection of serotonin in platelets . Br J Haematol . 2002;116:604–611
  146. Gordeuk VR , Stockton DW , Prchal JT . Congenital polycythemias/erythrocytoses . Haematologica . 2005;90:109–116
  147. Wasserman LR . The management of polycythemia vera . Br J Haematol . 1971;21:371–376
  148. Pearson TC , Botterill CA , Glass UH , Wetherley-Mein G . Interpretation of measured red cell mass and plasma volume in males with elevated venous PCV values . Scand J Haematol . 1984;33:68–74
  149. Fairbanks VF . Myeloproliferative disease: Polycythemia vera: The packed cell volume and the curious logic of the red cell mass . Hematology . 2000;4:381–395
  150. Berson SA , Yalow RS . The use of K42 or P32 labeled erythrocytes and I131 tagged human serum albumin in simultaneous blood volume determinations . J Clin Invest . 1952;31:572–580
  151. Lorberboym M , Rahimi-Levene N , Lipszyc H , Kim CK . Analysis of red cell mass and plasma volume in patients with polycythemia . Arch Pathol Lab Med . 2005;129:89–91
  152. Fairbanks VF , Tefferi A . Normal ranges for packed cell volume and hemoglobin concentration in adults (Relevance to ‘apparent polycythemia’) . Eur J Haematol . 2000;65:285–296
  153. Knutsen H . Polycythemia vera evaluation algorithm revisited . Mayo Clin Proc . 2004;79:430
  154. Johansson PL , Safai-Kutti S , Kutti J . An elevated venous haemoglobin concentration cannot be used as a surrogate marker for absolute erythrocytosis (A study of patients with polycythaemia vera and apparent polycythaemia) . Br J Haematol . 2005;129:701–705
  155. Chievitz E , Thiede T . Complications and causes of death in polycythemia vera . Acta Med Scand . 1962;172:513–523
  156. Gardner FH . Early approaches in the treatment of polycythemia vera . In:  Wasserman LR ,  Berk PD ,  Berlin NI editor. Polycythemia Vera and the Myeloproliferative Disorders . Philadelphia, PA: Saunders; 1995;p. 147–153
  157. Osgood EE . Leukaemogenic effect of ionising-irradiation treatment in polycythaemia . Lancet . 1964;67:967
  158. Modan B , Lilienfeld AM . Polycythemia vera and leukemia–The role of radiation treatment. A study of 1222 patients . Medicine (Baltimore) . 1965;44:305–344
  159. Holland PV , Menken M , Berlin NI . Autoimmune hemolytic anemia, acute leukemia, and carcinoma of the lung. Sodium phosphate P 32 treatment of polycythemia vera . Arch Intern Med . 1967;120:341–344
  160. Berk PD , Wasserman LR , Fruchtman SM , Goldberg JD . Treatment of polycythemia vera (A summary of clinical trials conducted by the Polycythemia Vera Study Group) . In:  Wasserman LR ,  Berk PD ,  Berlin NI editor. Polycythemia Vera and the Myeloproliferative Disorders . Philadelphia, PA: Saunders; 1995;p. 166–194
  161. Tartaglia AP , Goldberg JD , Berk PD , Wasserman LR . Adverse effects of antiaggregating platelet therapy in the treatment of polycythemia vera . Semin Hematol . 1986;23:172–176
  162. “Leukemia and Hematosarcoma” Cooperative Group, European Organization for Research on Treatment of Cancer (E.O.R.T.C.) . Treatment of polycythaemia vera by radiophosphorus or busulphan (A randomized trial) . Br J Cancer . 1981;44:75–80
  163. Najean Y , Rain JD . Treatment of polycythemia vera—The use of hydroxyurea and pipobroman in 292 patients under the age of 65 years . Blood . 1997;90:3370–3377
  164. Najean Y , Rain JD  The French Polycythemia Study Group . Treatment of polycythemia vera (Use of 32P alone or in combination with maintenance therapy using hydroxyurea in 461 patients greater than 65 years of age) . Blood . 1997;89:2319–2327
  165. Landolfi R , Marchioli R , Kutti J , et al.   Efficacy and safety of low-dose aspirin in polycythemia vera . N Engl J Med . 2004;350:114–124
  166. Fruchtman SM , Mack K , Kaplan ME , Peterson P , Berk PD , Wasserman LR . From efficacy to safety—A Polycythemia Vera Study Group report on hydroxyurea in patients with polycythemia vera . Semin Hematol . 1997;34:17–23
  167. Anagrelide Study Group . Anonymous: Anagrelide, a therapy for thrombocythemic states: Experience in 577 patients . Am J Med . 1992;92:69–76
  168. Silver RT . Interferon alfa (Effects of long-term treatment for polycythemia vera) . Semin Hematol . 1997;34:40–50
  169. Silver RT . Imatinib mesylate (Gleevec) reduces phlebotomy requirements in polycythemia vera . Leukemia . 2003;17:1186–1187
  170. Jurgens DJ , Moreno-Aspitia A , Tefferi A . Anagrelide-associated cardiomyopathy in polycythemia vera and essential thrombocythemia . Haematologica . 2004;89:1394–1395
  171. Elliott MA , Tefferi A . Interferon-alpha therapy in polycythemia vera and essential thrombocythemia . Semin Thromb Hemost . 1997;23:463–472
  172. Breccia M , Carmosino I , Russo E , Morano SG , Latagliata R , Alimena G . Early and tardive skin adverse events in chronic myeloid leukaemia patients treated with imatinib . Eur J Haematol . 2005;74:121–123
  173. Lin NU , Sarantopoulos S , Stone JR , et al.   Fatal hepatic necrosis following imatinib mesylate therapy . Blood . 2003;102:3455–3456
  174. Green AR , Campbell P , Buck G , et al.   The medical research council PT1 trial in essential thrombocythemia . Blood . 2004;104:6; (abstr)
  175. Wasserman LR , Balcerzak SP , Berk PD , et al.   Influence of therapy on causes of death in polycythemia vera . Trans Assoc Am Physicians . 1981;94:30–38
  176. Andreasson B , Lofvenberg E , Westin J . Management of patients with polycythaemia vera (Results of a survey among Swedish haematologists) . Eur J Haematol . 2005;74:489–495
  177. Streiff MB , Smith B , Spivak JL . The diagnosis and management of polycythemia vera in the era since the Polycythemia Vera Study Group (A survey of American Society of Hematology members’ practice patterns) . Blood . 2002;99:1144–1149
  178. Budde U , Schaefer G , Mueller N , et al.   Acquired von Willebrand’s disease in the myeloproliferative syndrome . Blood . 1984;64:981–985
  179. Tefferi A . Hydroxyurea vindicated (lack of drug leukemogenicity in polycythemia vera) . Curr Hematol Rep . 2005;4:211–212
  180. Griesshammer M , Bergmann L , Pearson T . Fertility, pregnancy and the management of myeloproliferative disorders . Baillieres Clin Haematol . 1998;11:859–874
  181. Passamonti F , Brusamolino E , Lazzarino M , et al.   Efficacy of pipobroman in the treatment of polycythemia vera (Long-term results in 163 patients) . Haematologica . 2000;85:1011–1018
  182. Tefferi A , Fonseca R . Selective serotonin reuptake inhibitors are effective in the treatment of polycythemia vera-associated pruritus . Blood . 2002;99:2627; (letter)
  183. de Wolf JT , Hendriks DW , Egger RC , Esselink MT , Halie MR , Vellenga E . Alpha-interferon for intractable pruritus in polycythaemia vera . Lancet . 1991;337:241
  184. Spivak JL . The optimal management of polycythaemia vera . Br J Haematol . 2002;116:243–254
  185. Cervantes F , Pereira A , Esteve J , et al.   Identification of ‘short-lived’ and ’long-lived’ patients at presentation of idiopathic myelofibrosis . Br J Haematol . 1997;97:635–640
  186. Gruppo Italiano Studio Policitemia . Polycythemia vera (The natural history of 1213 patients followed for 20 years) . Ann Intern Med . 1995;123:656–664
  187. Rector WG , Fortuin NJ , Conley CL . Non-hematologic effects of chronic iron deficiency. A study of patients with polycythemia vera treated solely with venesections . Medicine . 1982;61:382–389
  188. Messinezy M , Pearson TC , Prochazka A , Wetherley-Mein G . Treatment of primary proliferative polycythaemia by venesection and low dose busulphan (Retrospective study from one centre) . Br J Haematol . 1985;61:657–666
  189. Randi ML , Ruzzon E , Luzzatto G , Tezza F , Girolami A , Fabris F . Safety profile of hydroxyurea in the treatment of patients with Philadelphia-negative chronic myeloproliferative disorders . Haematologica . 2005;90:261–262
  190. Nand S , Messmore H , Fisher SG , Bird ML , Schulz W , Fisher RI . Leukemic transformation in polycythemia vera (analysis of risk factors) . Am J Hematol . 1990;34:32–36
  191. Elliott MA , Chen MG , Silverstein MN , Tefferi A . Splenic irradiation for symptomatic splenomegaly associated with myelofibrosis with myeloid metaplasia . Br J Haematol . 1998;103:505–511

PII: S0037-1963(05)00145-9

doi: 10.1053/j.seminhematol.2005.08.003

Seminars in Hematology
Volume 42, Issue 4 , Pages 206-220 , October 2005

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