Patterns of Ruxolitinib Therapy Failure and Its Management in Myelofibrosis: Perspectives of the Canadian Myeloproliferative Neoplasm Group

摘要

Primary myelofibrosis is a rare chronic myeloproliferative neoplasm (MPN) originating from the hematopoietic stem cell. Primary myelofibrosis is characterized by cytopenias, extramedullary hematopoiesis, megakaryocytic hyperplasia, marrow fibrosis, and systemic symptoms resulting from elevated levels of inflammatory and proangiogenic cytokines. A form of myelofibrosis indistinguishable from primary myelofibrosis can occur as part of the natural history of polycythemia vera and essential thrombocythemia, referred to as postpolycythemia vera or postessential thromobocytopenia myelofibrosis. The term myelofibrosis is used in this article to include primary myelofibrosis and myelofibrosis evolved from polycythemia vera or essential thrombocytopenia.