title: Elevated levels of basic fibroblast growth factor in megakaryocytes and platelets from patients with idiopathic myelofibrosis.
abstract: Idiopathic myelofibrosis, or agnogenic myeloid metaplasia, is a chronic myeloproliferative disorder characterized by clonal expansion and marrow fibrosis. Although marrow fibrosis appears to be a reactive process, it substantially contributes to impaired haemopoiesis. During the last few years the implication of megakaryocyte-derived growth factors in its pathogenesis has been documented. We previously reported increased expression of TGF-beta in patients with idiopathic myelofibrosis. In the present study we show that circulating megakaryocytic cells from such patients expressed high levels of basic fibroblast growth factor (bFGF). An increased expression of bFGF was also detected in patients' platelets. Under culture conditions, bFGF present in megakaryocytic cells was not exported into the medium. consistent with the fact that bFGF is devoid of a secretion peptide signal. Interestingly, this lack of bFGF secretion was observed in all patients but one, who was in an accelerated phase of the disease and presented an important percentage of circulating megakaryoblasts.
authors.affiliation: Unité 365 INSERM, Institut Curie, Section de Recherche, Paris, France.
authors.forename: M C
authors.initials: MC
authors.lastname: Martyré
authors.forename: M C
authors.initials: MC
authors.lastname: Le Bousse-Kerdiles
authors.forename: N
authors.initials: N
authors.lastname: Romquin
authors.forename: S
authors.initials: S
authors.lastname: Chevillard
authors.forename: V
authors.initials: V
authors.lastname: Praloran
authors.forename: J L
authors.initials: JL
authors.lastname: Demory
authors.forename: B
authors.initials: B
authors.lastname: Dupriez
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D001792:Blood Platelets / Q000378:metabolism*; D005260:Female; D016222:Fibroblast Growth Factor 2 / Q000378:metabolism*; D006801:Humans; D008297:Male; D008533:Megakaryocytes / Q000378:metabolism*; D008875:Middle Aged; D055728:Primary Myelofibrosis / Q000378:metabolism*; D012333:RNA, Messenger / Q000378:metabolism; D016212:Transforming Growth Factor beta / Q000378:metabolism
chemical_list: D012333:RNA, Messenger; D016212:Transforming Growth Factor beta; D016222:Fibroblast Growth Factor 2