Long-term bone marrow cultures (LTBMC) from essential thrombocythemia (ET) patients with or without JAK2617V>F mutation

Leuk Res. 2008 Oct;32(10):1593-6. doi: 10.1016/j.leukres.2008.01.013. Epub 2008 Mar 5.

Abstract

Approximately half of essential thrombocythemia (ET) patients and almost all with polycythemia vera (PV) bear the activating JAK2617V>F point mutation, which arises at the multipotent haemopoietic progenitor cell level. Although ET is mainly characterized by megacaryocyte proliferation, the cases that are positive for the JAK2617V>F mutation also show increased bone marrow cellularity and higher erythrocyte and granulocyte counts. After establishing short- and long-term bone marrow cultures we found that the frequency of committed haemopoietic progenitors in the bone marrow, was not increased in JAK2617V>F positive ET compared to the negative ones, whereas in long-term cultures (LTBMC) JAK2617V>F positive ET display a growth pattern more similar to that observed in LTBMC produced by PV marrow cells. Our data support the notion that JAK2617V>F positive ET and PV represents a continuum spectrum of alterations within the same disease.

MeSH terms

  • Amino Acid Substitution
  • Cells, Cultured
  • Hematopoiesis / genetics*
  • Hematopoietic Stem Cells / pathology
  • Humans
  • Janus Kinase 2 / genetics*
  • Point Mutation*
  • Polycythemia Vera / pathology
  • Thrombocythemia, Essential / genetics*
  • Thrombocythemia, Essential / pathology

Substances

  • JAK2 protein, human
  • Janus Kinase 2