18. Myeloproliferative Neoplasms and Myelodysplastic Syndromes

  1. Alvin H. Schmaier MD and
  2. Hillard M. Lazarus MD, FACP
  1. Gabriela Motyckova MD, PhD and
  2. Richard M. Stone MD

Published Online: 22 SEP 2011

DOI: 10.1002/9781444345254.ch18

Concise Guide to Hematology

Concise Guide to Hematology

How to Cite

Motyckova, G. and Stone, R. M. (2011) Myeloproliferative Neoplasms and Myelodysplastic Syndromes, in Concise Guide to Hematology (eds A. H. Schmaier and H. M. Lazarus), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781444345254.ch18

Editor Information

  1. Case Western Reserve University, University Hospitals Case Medical Center, Cleveland, OH, USA

Author Information

  1. Dana-Farber Cancer Institute, Boston, MA, USA

Publication History

  1. Published Online: 22 SEP 2011
  2. Published Print: 4 NOV 2011

ISBN Information

Print ISBN: 9781405196666

Online ISBN: 9781444345254

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Keywords:

  • myeloproliferative neoplasm;
  • myelodysplastic syndrome;
  • chronic myeloid leukemia;
  • polycythemia vera;
  • essential thrombocytosis;
  • primary myelofibrosis

Summary

The myeloproliferative neoplasms (MPN) and myelodysplastic syndromes (MDS) are clonal diseases of hematopoietic stem cells causing abnormal proliferation and/or ineffective production of blood cells. The treatment of MPNs including polycythemia vera and essential thrombocytosis focuses on control of the peripheral blood counts and reducing complications such as bleeding and thrombosis. In addition to these therapeutic considerations, the treatment of primary myelofibrosis also aims to reduce the side effects of extramedullary hematopoiesis. In chronic myeloid leukemia, the discovery of imatinib and other tyrosine kinase inhibitors led to the successful management of this disease in a large percentage of patients. Myelodysplastic syndromes (MDS) are heterogeneous diseases where treatment decisions are based on baseline risk and therapies range from supportive care to allogeneic stem cell transplantation. Both MPN and MDS carry a risk of transformation into acute leukemia.