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Polycythemia vera. A clinical study of 141 patients

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Summary

The clinical course of 141 unselected patients (64 m, 77 f, median age 59) with polycythemia vera (PV), treated during the period 1967 to 1986 was analyzed to study prognostic factors and the correlation between treatment strategies and complication rates. Therapy was performed according to a prospectively defined treatment protocol. Primary control of the disease was achieved by phlebotomy. Marrow suppression by radioactive phosphorus or low dose busulphan was used only as a second-line therapy or to lower high platelet counts. The clinical course of the patients was characterized by a low rate of acute leukemia (4%) and a high rate of thromboembolic complications (40%). Myelofibrosis developed in 17 patients (12%). Median survival of the patients was 9.4 years. The prognostic influence of several parameters at the time of diagnosis was tested: age, sex, spleen size, percentage of blood blasts + promyelocytes, leucocyte count, platelet count, hemoglobin, hematocrit, reticulocyte count and the values of the lactatdehydrogenase (LDH) and the alkaline neutrophil phosphatase (ANP) all had no significant influence on the length of survival. The prognosis of PV patients with atypical disease presentation at diagnosis was not different from patients with typical disease.

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References

  1. Adamson JW, Fialkow PJ, Murphy S (1976) Polycythemia vera: Stem cell and probable clonal origin of the disease. N Engl J Med 295: 913–916

    Google Scholar 

  2. Anger BR, Carbonell F, Braunger I, Heinze B, Gutensohn W, Thiel E, Heimpel H (1988) Blast crisis of Philadelphia chromosome-positive chronic myelocytic leukemia (CML). Blut 57: 131–137

    Google Scholar 

  3. Berger R, Bernheim A, Le Coniat M, Vecchione D, Flandrin G, Dresch C, Najean Y (1984) Chromosome studies in polycythemia vera patients. Cancer Genet Cytogenet 12: 217

    Google Scholar 

  4. Berk PD, Goldberg JD, Silverstein MN et al. (1981) Increased incidence of acute leukemia in polycythemia vera associated with chlorambucil therapy. N Engl J Med 304: 441–447

    Google Scholar 

  5. Berk PD, Goldberg JD, Silverstein MN (1986) Therapeutic recommendations in polycythemia vera based on Polycythemia Vera Study Group protocols. Semin Hematol 23: 132–143

    Google Scholar 

  6. Berlin N (1975) Diagnosis and classification of the polycythemias. Semin Hematol 12: 339–351

    Google Scholar 

  7. Donehower RC (1982) Hydroxyurea. In: Chabner P (ed) Pharmacological principles of cancer treatment. Philadelphia: Saunders, pp 269–275

    Google Scholar 

  8. Donovan PB, Kaplan ME, Goldberg JD (1984) Treatment of polycythemia vera with hydroxyurea. Am J Hematol 17: 329–334

    Google Scholar 

  9. Ellis JT, Peterson P, Geller SA, Rappaport H (1986) Studies of the bone marrow in polycythemia vera and the evolution of myelofibrosis and second hematologic malignancies. Semin Hematol 23: 144–155

    Google Scholar 

  10. European Organization for Research on Treatment of Cancer “Leukemia and Hematosarcoma” Cooperative Group (1981) Treatment of polycythemia vera by radioactive phosphorus or busulfan. Br J Cancer 44: 74–80

    Google Scholar 

  11. Fialkow PJ (1980) Clonal and stem cell origin of blood cell neoplasms. In: Lobue J, Gordon AS, Silber R (eds) Contemporary hematology oncology, vol. 1. Plenum, New York

    Google Scholar 

  12. Hehlmann R, Anger BR, Messerer D, Zankovitch R, Bergmann L, Kolb HJ, Meyer P, Essers U, Queisser U, Vaupel H, Walter F, Hossfeld DK, Zimmermann R, Heiss F, Mende S, Tigges FJ, Kleeberg UR, Pralle H, Kayser W, Tichelli A, Faulhaber JD, Raeth U, Schubert H, Bross K, Schlag R, Schmid L, Weissenfels I, Heinze B, Georgii A, Queisser W, Heimpel H (1988) Randomized study on the treatment of chronic myeloid leukemia (CML) in chronic phase with busulfan versus hydroxyurea versus interferon-alpha. Blut 56: 87–91

    Google Scholar 

  13. Ikkala E, Rapola J, Kotilainen M (1967) Polycythemia vera and myelofibrosis. Scand J Haematol 4: 453–464

    Google Scholar 

  14. Kaplan ME, Mack K, Goldberg JD, Donovan PB, Berk PD, Wassermann LR (1986) Long-term management of polycythemia vera with hydroxyurea: A progress report. Semin Hematol 23: 167–171

    Google Scholar 

  15. Landaw SA (1986) Acute leukemia in polycythemia vera. Semin Hematol 23: 156–165

    Google Scholar 

  16. Laszlo J (1975) Myeloproliferative disorders (MPD): myelofibrosis, myelosclerosis, extramedullary hematopoiesis, undifferentiated MPD, and hemorrhagic thrombocythemia. Semin Hematol 12: 409–432

    Google Scholar 

  17. Messinezy M, Pearson TC, Prochazka A, Wetherley Mein G (1985) Treatment of primary proliferative polycythaemia by venesection and low dose busulphan: Retrospective study from one center. Br J Haematol 61: 657–666

    Google Scholar 

  18. Miller JB, Tests JR, Lindgren V, Rowley JD (1985) The pattern and clinical significance of karyotypic abnormalities in patients with idiopathic and postpolycythemic myelofibrosis. Cancer 55: 582–591

    Google Scholar 

  19. Modan B, Lilienfeld AM (1965) Polycythemia vera and leukemia — the role of radiation treatment: A study of 1,222 patients. Medicine 44: 305–344

    Google Scholar 

  20. Murphy S, Iland H, Rosenthal D, Laszlo J (1986) Essential thrombocythemia: An interim report from the Polycythemia Vera Study Group. Semin Hematol 23: 177–182

    Google Scholar 

  21. Najean Y, Arrago JP, Rain JD, Dresch C (1984) The “spent” phase of polycythemia vera: Hypersplenism in the absence of myelofibrosis. Br J Haematol 56: 163–170

    Google Scholar 

  22. Osgood EE (1965) Polycythemia vera: Age relationship and survival. Blood 26: 243–256

    Google Scholar 

  23. Osler W (1903) Chronic cyanosis with polycythemia and enlarged spleen: A new clinical entity. Am J Med Sci 126: 187–201

    Google Scholar 

  24. Pearson TC (1987) Rheology of the absolute polycythaemias. In: Lowe GDO (ed) Clinical haematology, vol. 1. Blood rheology and hyperviscosity syndromes. Bailliere Tindall, London, pp 637–664

    Google Scholar 

  25. Perkins J, Israels MCG, Wilkinson JF (1964) Polycythaemia vera: Clinical studies on a series of 127 patients managed without radiation therapy. J Med 33: 499–518

    Google Scholar 

  26. Silverstein MN (1974) Postpolycythemia myeloid metaplasia. Arch Intern Med 134: 113–115

    Google Scholar 

  27. Silverstein MN (1976) The evolution into and the treatment of late stage polycythemia vera. Semin Hematol 13: 79–84

    Google Scholar 

  28. Swolin B, Weinfeld A, Westin J (1988) A prospective long-term cytognetic study in polycythemia vera in relation to treatment and clinical course. Blood 71: 386–395

    Google Scholar 

  29. Szur L, Lewis SM (1966) The haematological complications of polycythaemia vera and treatment with radioactive phosphorus. Br J Radiol 39: 122–130

    Google Scholar 

  30. Tartaglia A, Goldberg JD, Berk PD, Wassermann LR (1986) Adverse effects of antiaggregting platelet therapy in the treatment of polycythemia vera. Semin Hematol 23: 172–176

    Google Scholar 

  31. Testa J, Karnofsky JR, Rowley JD, Baron JM, Vardiman JW (1981) Karyotypic patterns and their clinical significance in polycythemia vera. Am J Hematol 11: 29

    Google Scholar 

  32. Tubiana M, Flamant R, Attie E, Hayat M (1968) A study of hematological complications occuring in patients with polycythemia vera treated with P 32. Blood 32: 536–548

    Google Scholar 

  33. Turk W (1904) Beitrag zur Kenntnis des Symptomenbildes Polycythaemie mit Milztumor and Zyanose. Wien Klin Wochenschr 17: 153–160

    Google Scholar 

  34. Valla D, Casadevall N, Lacombe C (1985) Primary myeloproliferative disorders and hepatic vein thrombosis: A prospective study of erythroid colony formation in vitro in 20 patients with Budd-Chiari syndrome. Ann Intern Med 103: 329–334

    Google Scholar 

  35. Vaquez H (1982) Sur une forme speciale de cyanose s'accompanant d'hyperglobulie excessive et persistente. Compt Rend Soc De Biol 44: 384–388

    Google Scholar 

  36. Videbaek A (1950) Polycythemia vera, course and prognosis. Acta Med Scand 138: 179–187

    Google Scholar 

  37. Wassermann LR, Balcerzak SP, Berlin NI et al. (1981) Influence of therapy on causes of death in polycythemia vera. Trans Assoc Am Physicians 94: 30–38

    Google Scholar 

  38. Weinfeld A, Westin J, Ridell B, Swolin B (1977) Polycythemia vera terminating in acute leukemia. Scand J Haematol 19: 255–272

    Google Scholar 

  39. Zankovich R, Thiele J, Fiessler WD, Fischer R, Diehl V (1987) Prognostic parameters in polycythemia vera rubra. Correlations of clinical and histomorphological features in 60 patients. Med Klinik 82: 889–894

    Google Scholar 

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Authors and Affiliations

  1. Division of Hematology, Department of Internal Medicine, Ulm University Hospital, Robert-Koch-Strasse 8, D-7900, Ulm, Federal Republic of Germany

    B. Anger, U. Haug, R. Seidler & H. Heimpel

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  1. B. Anger
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  2. U. Haug
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  3. R. Seidler
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  4. H. Heimpel
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Anger, B., Haug, U., Seidler, R. et al. Polycythemia vera. A clinical study of 141 patients. Blut 59, 493–500 (1989). https://doi.org/10.1007/BF00329494

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