Article ID Journal Published Year Pages File Type
5524374 Biology of Blood and Marrow Transplantation 2017 8 Pages PDF
Abstract

•Treatment options for myelofibrosis include symptom management, JAK inhibitors, and allogeneic stem cell transplantation (HCT).•Outcomes of HCT in patients with myelofibrosis are reviewed, and HCT is recommended for eligible patients with intermediate-2 or high-risk disease.•Pretransplantation factors such as donor selection and conditioning regimens are reviewed•Post-HCT challenges of graft failure, relapse, donor lymphocyte infusion, and graft-versus-host disease are discussed.

Myeloproliferative neoplasm (MPN) is a category in the World Health Organization classification of myeloid tumors. BCR-ABL1-negative MPN is a subcategory that includes primary myelofibrosis (MF), post-essential thrombocythemia MF, and post-polycythemia vera MF. These disorders are characterized by stem cell-derived clonal myeloproliferation. Clinically, these diseases present with anemia and splenomegaly and significant constitutional symptoms such as severe fatigue, symptoms associated with an enlarged spleen and liver, pruritus, fevers, night sweats, and bone pain. Multiple treatment options may provide symptom relief and improved survival; however, allogeneic stem cell transplantation (HCT) remains the only potentially curative option. The decision for a transplant is based on patient prognosis, age, comorbidities, and functional status. This review describes the recent data on various peritransplantation factors and their effect on outcomes of patients with MF and new therapeutic areas, such as the use and timing of Janus kinase inhibitors with HCT and gives overall conclusions from the available data in the published literature.

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