Essential thrombocythemia - Update on pathogenesis and therapy

G. Lugassy

Research output: Contribution to journalReview articlepeer-review

2 Scopus citations

Abstract

Essential thrombocythemia (ET) is a clonal myeloproliferative disorder of the multipotent hematopoietic stem cell. ET is characterized by a persistent increase in circulating platelets and an excessive proliferation of megakaryocytes in the bone marrow. The culture of peripheral blood or bone marrow from ET patents gives rise to an increased number of colony-forming units composed of megakaryocytes, suggesting that the pathological clone has a preferential responsiveness to regulatory factors that favor its differentiation into the megakaryocytic-platelet lineage. The newly described TPO-c-mpl system, is an important physiological regulator of megakaryocyte and platelet production. Although the TPO-c-mpl system is dysregulated in ET, there is no evidence that it is directly linked to the pathogenesis of the disease. Diagnosis of ET is one of exclusion, and no clinical or laboratory criteria can predict the occurrence of thrombohemorrhagic complications. Therefore, the main challenge in ET is to define the population of patients that could benefit from platelet-lowering therapy. The introduction of α- interferon and of anagrelide, a megakaryocyte-platelet specific agent, represent major advances in therapy of ET patients.

Original languageEnglish
Pages (from-to)57-59
Number of pages3
JournalCancer Journal
Volume11
Issue number2
StatePublished - 1 Jan 1998

Keywords

  • Anagrelide
  • Essential thrombocythemia
  • Interferon
  • Thrombopoietin
  • Thrombosis
  • Von Willebrand factor
  • mpl receptor

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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