Myeloproliferative Disorders: Polycythemia Vera, Essential Thrombocythemia, and Idiopathic Myelofibrosis/Agnogenic Myeloid Metaplasia

  • H. J. Iland
  • J. Laszlo
Part of the UICC Current Treatment of Cancer book series (1360)


In 1951 William Dameshek speculated that under certain conditions hematopoietic cells (precursors of erythrocytes, granulocytes, and platelets), as well as fibroblasts, proliferated “en masse” within the bone marrow in response to a myelostimulatory factor. This factor, said Dameshek, appeared also to activate dormant embryonal hematopoietic tissue in the liver and spleen. These processes resulted in a spectrum of clinicopathologic entities which Dameshek frred to as the myeloproliferative syndromes. The concept of myeloproliferative syndrome, which was well grounded in studies of hematopathology by earlier workers, has gained widespread acceptance, although more recent knowledge about hematopoiesis has necessitated certain modifications to the original hypothesis.


Disseminate Intravascular Coagulation Polycythemia Vera Essential Thrombocythemia Splenic Infarction Extramedullary Hematopoiesis 
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Further Reading

Polycythemia Vera

  1. Dameshek W (1951) Some speculations on the myeloproliferative syndromes. Blood 6: 372–375PubMedGoogle Scholar
  2. Murphy S (1983) Polycythemia vera. In: Williams WJ, Beutler E, Erslev AJ, Lichtman MA (eds) Hematology, 3rd ed. McGraw-Hill, New York, pp 185–196Google Scholar
  3. Silverstein MN (1974) Postpolycythemia myeloid metaplasia. Arch Intern Med 134: 113–115PubMedCrossRefGoogle Scholar
  4. Weinreb NJ, Shih C-F (1975) Spurious polycythemia. Semin Hematol 12: 397–407PubMedGoogle Scholar
  5. Ellis JT, Peterson P (1979) The bone marrow in polycythemia vera. Pathol Annu 14: 383–403PubMedGoogle Scholar
  6. Berlin NI (1975) Diagnosis and classification of the polycythemias. Semin Hematol 12: 339–351PubMedGoogle Scholar
  7. Loeb V Jr (1975) Treatment of polycythemia vera. Clin Haematol 4: 441–456PubMedGoogle Scholar
  8. Wasserman LR, Balcerzak SP, Berk PD et al. (1981) Influence of therapy on causes of death in polycythemia vera. Trans Assoc Am Physicians 94: 30–38PubMedGoogle Scholar
  9. Rowley JD (1976) The role of cytogenetics in hematology. Blood 48: 1–7PubMedGoogle Scholar

Essential Thrombocythemia

  1. Gunz FW (1960) Hemorrhagic thrombocythemia: a critical review. Blood 15: 706–723PubMedGoogle Scholar
  2. Ozer FL, Truax WE, Miesch DC, Levin WC (1960) Primary hemorrhagic thrombocythemia. Am J Med 28: 807–823PubMedCrossRefGoogle Scholar
  3. Hand HJ, Laszlo J, Peterson P et al. (1983) Essential thrombocythemia: clinical and laboratory characteristics at presentation. Trans Assoc Am Physicians 96: 165–174Google Scholar
  4. Hoagland HC, Silverstein MN (1978) Primary thrombocythemia in the young patient. Mayo Clin Proc 53: 578–580PubMedGoogle Scholar
  5. Kessler CM, Klein HG, Havlik RJ (1982) Uncontrolled thrombocytosis in chronic myeloproliferative disorders. Br J Haematol 50: 157–167PubMedCrossRefGoogle Scholar
  6. Jabaily J, Iland HJ, Laszlo J et al. (1983) Neurologic manifestations of essential thrombocythemia. Ann Intern Med 99: 513–518PubMedGoogle Scholar
  7. Murphy S, Rosenthal DS, Weinfeld A et al. (1982) Essential thrombocythemia: response during first year of therapy with melphalan and radioactive phosphorus: a Polycythemia Vera Study Group report. Cancer Treat Rep 66: 1495–1500PubMedGoogle Scholar
  8. Younger J, Umlas J (1978) Rapid reduction of platelet count in essential hemorrhagic thrombocythemia by discontinuous flow plateletpheresis. Am J Med 64: 659–661PubMedCrossRefGoogle Scholar
  9. Zucker S, Mielke CH (1972) Classification of thrombocytosis based on platelet function tests: correlation with hemorrhagic and thrombotic complications. J Lab Clin Med 80: 385–394PubMedGoogle Scholar
  10. Preston FE, Emmanuel IG, Winfield DA, Malia RG (1974) Essential thrombocythaemia and peripheral gangrene. Br Med J 3: 548–552PubMedCrossRefGoogle Scholar

Idiopathic Myelofibrosis/Agnogenic Myeloid Metaplasia

  1. Ward HP, Block MH (1971) The natural history of agnogenic myeloid metaplasia (AMM) and a critical evaluation of its relationship with the myeloproliferative syndrome. Medicine 50: 357–420PubMedCrossRefGoogle Scholar
  2. Rosenthal DS, Moloney WC (1969) Myeloid metaplasia: a study of 98 cases. Postgrad Med 45: 136–142PubMedGoogle Scholar
  3. Laszlo J (1975) Myeloproliferative disorders (MPD): myelofibrosis, myelosclerosis, extramedullary hematopoiesis, undifferentiated MPD and hemorrhagic thrombocythemia. Semin Hematol 12: 409–432PubMedGoogle Scholar
  4. Laszlo J, Huang AT (1977) Diagnosis and management of myeloproliferative disorders. Curr Probl Cancer 2 (1)Google Scholar
  5. Laszlo J (1983) Anemia associated with marrow infiltration. In: Williams WJ, Beutler, E, Erslev AJ, Lichtman MA (eds) Hematology, 3rd ed. McGraw-Hill, New York pp 528–532Google Scholar
  6. Castro-Malaspina H, Moore MAS (1982) Pathophysiological mechanisms operating in the development of myelofibrosis: role of megakaryocytes. Nouv Rev Hematol 24: 221–226Google Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1986

Authors and Affiliations

  • H. J. Iland
  • J. Laszlo

There are no affiliations available

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