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The Pathogenesis, Diagnosis, and Treatment of Polycythaemia Vera

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Neoplastic Diseases of the Blood

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Abstract

Polycythaemia vera (PV) was first described in 1892 by Vazquez [1] in a case report describing a patient with ruddy cyanosis, splenomegaly, and an increased red cell count not associated with a congenital form of heart disease. A decade later in 1903 it was defined more clearly by Osler [2], and the disease then became known as Vasquez–Osler disease until this eponymous term was superseded by PV. PV together with essential thrombocythaemia (ET) and primary myelofibrosis (PMF) (Chap. 12) represent an overlapping spectrum of clonal haematological disorders called the human myeloproliferative neoplasms (MPN). The MPNs were first grouped together, along with chronic myeloid leukaemia (CML), by Dameshek in his seminal paper of 1951 [3]. CML is now generally considered as a distinct entity, but shares several features with the other MPNs. All of these disorders result from acquired genetic changes in the haematopoietic stem cell compartment and are characterized by proliferation of various cells of the myeloid lineages. They also all share the propensity to develop into acute myeloid leukaemia (AML), albeit with varying incidence, and, as will be described next, the majority demonstrate abnormalities of intracellular signalling. Taking all of these characteristics together, the MPN and CML therefore provide in vivo model systems to study the multistep development of AML. In addition, as MPNs are associated with full terminal differentiation of myeloid lineages, they allow the study of the effects of oncogenic mutations on normal myeloid homeostasis before this is complicated by cooperating mutations which block differentiation.

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Acknowledgement

The authors also acknowledge Dr Anthony Bench and Professor Tony Green. Dr Bench and Professor Green were ­co-authors on a previous version of this chapter, whose format provided a loose framework for this significant update.

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  1. Department of Hematology, Cambridge Institute for Medical Research, Hills Road, Cambridge, CB2 0XY, UK

    Mark A. Dawson M.B.B.S (Hons), B.Med.Sc., F.R.A.C.P., F.R.C.P.A., Ph.D. & Brian J. P. Huntly M.B.Ch.B., M.R.C.P., Ph.D., F.R.C.Path

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  1. Mark A. Dawson M.B.B.S (Hons), B.Med.Sc., F.R.A.C.P., F.R.C.P.A., Ph.D.
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  2. Brian J. P. Huntly M.B.Ch.B., M.R.C.P., Ph.D., F.R.C.Path
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Correspondence to Brian J. P. Huntly M.B.Ch.B., M.R.C.P., Ph.D., F.R.C.Path .

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  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

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Dawson, M.A., Huntly, B.J.P. (2013). The Pathogenesis, Diagnosis, and Treatment of Polycythaemia Vera. 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_11

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