The pathogenesis of chronic myeloproliferative diseases.

Chronic myeloproliferative disorders are operationally classified to include essential thrombocythemia, polycythemia vera, and agnogenic myeloid metaplasia. In most cases, clonal hematopoiesis, involving all 3 myeloid lineages, can be demonstrated. However, the underlying molecular lesions that are responsible for disease initiation and progression remain elusive. There are ongoing efforts to clarify the pathogenetic role of cytokines, bone marrow stromal cells and molecules, and intracellular aberrations in either signal transduction or apoptosis. This review discusses some of the current and past observations regarding the pathogenesis of chronic myeloproliferative disorders.