Interferon-α therapy in polycythemia vera and essential thrombocythemia

Michelle A. Elliott, Ayalew Tefferi

Research output: Contribution to journalArticle

74 Citations (Scopus)

Abstract

Essential thrombocythemia (ET) and polycythemia vera (PV) are chronic clonal myeloid disorders that originate from the multipotential hematopoietic stem cell. They are characterized, respectively, by excessive thrombocytosis and erythrocytosis, a high incidence of thrombohemorrhagic events, vasomotor symptoms, and an inherent tendency to undergo leukemic transformation. Current standard therapies to control the excess accumulation of myeloid cells and to provide symptomatic relief carry either a persistent risk of thrombosis, as in the case of phlebotomy, or, in the case of hydroxyurea, the potential for inducing leukemia. None alter the natural history of these diseases. Interferon-α has been shown to have potent antiproliferative effects on the hematopoietic stem cells and bone marrow fibroblasts and, as a result, has received much attention as a therapeutic agent for chronic myeloproliferative disorders. The ability of interferon-α to induce hematologic and cytogenetic remission in chronic phase chronic granulocytic leukemia has further increased interest in this agent. Interferon-α has shown therapeutic activity in PV and ET, as demonstrated in multiple small studies and single-arm trials reviewed in this article. Reported beneficial effects include the ability to control excessive erythrocytosis and thrombocytosis and such disease-related features as vasomotor symptoms, pruritus, and splenomegaly. Recent reports of cytogenetic remission and reversal of bone marrow fibrosis after interferon therapy are of interest. Advantages over current therapeutic standards include lack of known leukemogenic and teratogenic effects and the potential to alter the underlying course of disease. Nevertheless, none of the information available allows definite therapeutic recommendations for the use of interferon-α in PV or ET. The available data support the need for randomized controlled trials comparing interferon-α with standard therapy.

Original languageEnglish (US)
Pages (from-to)463-472
Number of pages10
JournalSeminars in Thrombosis and Hemostasis
Volume23
Issue number5
StatePublished - 1997

Fingerprint

Essential Thrombocythemia
Polycythemia Vera
Interferons
Thrombocytosis
Polycythemia
Hematopoietic Stem Cells
Cytogenetics
Therapeutics
Leukemia, Myeloid, Chronic Phase
Myeloproliferative Disorders
Primary Myelofibrosis
Phlebotomy
Hydroxyurea
Splenomegaly
Therapeutic Uses
Myeloid Cells
Pruritus
Leukemia
Thrombosis
Randomized Controlled Trials

Keywords

  • Essential thrombocythemia
  • Pathogenesis
  • Polycythemia vera
  • Side effects
  • Treatment

ASJC Scopus subject areas

  • Hematology

Cite this

Interferon-α therapy in polycythemia vera and essential thrombocythemia. / Elliott, Michelle A.; Tefferi, Ayalew.

In: Seminars in Thrombosis and Hemostasis, Vol. 23, No. 5, 1997, p. 463-472.

Research output: Contribution to journalArticle

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