Thrombocytopenia in patients with myelofibrosis: management options in the era of JAK inhibitor therapy

Abstract

Myelofibrosis (MF), either appearing de novo (primary MF, PMF) or after a previous diagnosis of essential thrombocythemia or of polycythemia vera, is a progressive disease burdened by symptomatic splenomegaly, debilitating systemic symptoms, ineffective hematopoiesis, and overall reduced survival. Patients often present worsening cytopenias, including thrombocytopenia, secondary to progression of the disease as well as to cytoreductive treatment. Patients with MF and thrombocytopenia have few therapeutic options and there is limited information regarding the management of disease in these settings. This article reviews current evidence for the management of patients with MF and thrombocytopenia, in the era of JAK inhibitors.

Keywords:

• Myelofibrosis
• prognosis
• JAK inhibitors
• ruxolitinib
• thrombocytopenia

Acknowledgments

We thank Ray Hill, an independent medical writer, who provided English-language editing and journal styling prior to submission on behalf of Health Publishing and Services Srl. This assistance was funded by Novartis Farma, Italy.

Disclosure statement

GB had participated at advisory boards for Novartis, Celgene and Amgen. EME had participated at advisory boards for Novartis. AR had participated at advisory boards for Novartis, Sanofi and Alexion. PG and MM report no conflict of interest.