title: Mixed myelodysplastic syndrome and myeloproliferative disorder with bone marrow and pulmonary fibrosis: the role of megakaryocytes.
abstract: The case of an 80-year-old woman displaying myelodysplastic syndrome evolving into a myeloproliferative disorder with myelofibrosis and pulmonary fibrosis, is reported. This case is characterized by an initial presentation of a myelodysplastic syndrome with normal karyotype and moderate fibrosis, its evolution towards a myeloproliferative disorder with myelofibrosis and the worsening of pulmonary fibrosis in parallel to the acceleration of the myeloproliferative disorder and myelofibrosis. These features and the high concentration of plasma platelet factor-4 suggest a role of megakaryocyte/platelet degranulation in the development of fibrosis.
authors.affiliation: Service d'Angio-Hématologie Clinique, Hôpital Lariboisière, Paris, France.
authors.forename: Sophie
authors.initials: S
authors.lastname: Rosenstingl
authors.forename: Jean-Philippe
authors.initials: JP
authors.lastname: Brouland
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Zini
authors.forename: Gérard
authors.initials: G
authors.lastname: Tobelem
authors.forename: Evelyne
authors.initials: E
authors.lastname: Dupuy
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D015550:Cell Degranulation; D005260:Female; D006801:Humans; D008533:Megakaryocytes / Q000473:pathology* / Q000502:physiology*; D009190:Myelodysplastic Syndromes / Q000150:complications*; D009196:Myeloproliferative Disorders / Q000209:etiology* / Q000473:pathology; D010978:Platelet Factor 4 / Q000378:metabolism; D055728:Primary Myelofibrosis / Q000209:etiology* / Q000473:pathology; D011658:Pulmonary Fibrosis / Q000209:etiology* / Q000473:pathology; D013997:Time Factors
chemical_list: D010978:Platelet Factor 4