Thrombopoietin receptor activation, thrombopoietin mimetic drugs, and hereditary thrombocytosis: remarks on bone marrow fibrosis

Luciana Teofili, Maurizio Martini, Luigi Maria Larocca, Lorenza Torti, Tonia Cenci, Giuseppe Leone, F Giona, R Foa'

Risultato della ricerca: Contributo in rivistaArticolo in rivista

6 Citazioni (Scopus)

Abstract

Tpo mimetics romiplostim and eltrombopag stimulate megakaryocytopoiesis by mechanisms similar to those respondible for hereditary thrombocytosis.In particular, eltrombopag, a nonpeptide Tpo mimetic, binds to MPL in its transmembrane region and directly activates the signal transduction pathway. Thus, chronic eltrombopag administration reminds the constitutive activation of MPL as observed in patients with MPL germline muatations. Since these patients usually develop with aging bone marrow fibrosis, and given that these drugs have to be lifelong administered, concern emerged when it was noticed that some patients with immune thrombocytopenia (ITP) receiving romiplostin developed bone marrow fibrosis.
Lingua originaleEnglish
pagine (da-a)e317-e317-8
RivistaJournal of clinical oncology : official journal of the American Society of Clinical Oncology
Volume28
DOI
Stato di pubblicazionePubblicato - 2010

Keywords

  • Humans
  • Hydrazines
  • Mutation
  • Primary Myelofibrosis
  • Receptors, Fc
  • Receptors, Thrombopoietin
  • Recombinant Fusion Proteins
  • Thrombocytopenia
  • Thrombocytosis
  • Thrombopoietin
  • Transcriptional Activation

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