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Mixed Myelodysplastic Syndrome and Myeloproliferative Disorder with Bone Marrow and Pulmonary Fibrosis: The Role of Megakaryocytes

Authors
  • Rosenstingl, Sophie
  • Brouland, Jean-Philippe
  • Zini, Jean-Marc
  • Tobelem, Gérard
  • Dupuy, Evelyne
Type
Published Article
Journal
Acta Haematologica
Publisher
S. Karger AG
Publication Date
Apr 01, 2003
Volume
109
Issue
3
Pages
145–149
Identifiers
DOI: 10.1159/000069290
PMID: 12714825
Source
Karger
Keywords
License
Green
External links

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.

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