TGF-β and Megakaryocytes in the Pathogenesis of Myelofibrosis in Myeloproliferative Disorders

Abstract

Myeloproliferative disorders are clonal disorders of the hematopoietic stem cell and comprise a spectrum of more or less well-defined clinical entities: polycythaemia vera, chronic myeloid leukemia, essential thrombocythaemia, and agnogenic myeloid metaplasia. Myelofibrosis, which contributes substantially to the impaired hematopoiesis, is commonly observed in myeloproliferative disorders but it represents the criterion of agnogenic myeloid metaplasia also termed idiopathic myelofibrosis. Although progress has been made in the elucidation of the pathogenesis of myelofibrosis, it still remains unclear. The aim of this review is to adress the new insights that outline the potential role of TGF-β in the promotion of myelofibrosis, through its release from megakaryocytes/platelets, particularly in agnogenic myeloid metaplasia.

Key Words:

• myelofibrosis
• myeloproliferative disorders
• megakaryocytes
• TGF-β