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Megakaryoblastic transformation of myelofibrosis with expression of the c-sis oncogene.

Authors
Type
Published Article
Journal
Scandinavian journal of haematology
Publication Date
Volume
36
Issue
2
Pages
186–193
Identifiers
PMID: 2422734
Source
Medline
License
Unknown

Abstract

We describe a case of primary myelofibrosis which terminated in an acute megakaryoblastic leukaemia with massive marrow fibrosis and osteosclerosis. The megakaryocyte lineage of the terminal phase was confirmed by ultrastructural and surface marker studies of the blast cells. The leukaemic phase was associated with the presence of large numbers of progressively more immature megakaryocyte progenitors in the peripheral blood. The expression of c-sis mRNA in these blast cells was significantly higher than in normal mononuclear cells. Activation of the c-sis protooncogene leading to increased production of platelet-derived growth factor could be related to the progressive fibrosis observed.

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