title: Prognostication in MF: from CBC to cytogenetics to molecular markers.
abstract: Myelofibrosis (MF) is a clonal stem cell disorder characterized by ineffective erythropoiesis and extramedullary hematopoiesis leading to progressive bone marrow failure, severe anemia, constitutional symptoms, hepatosplenomegaly, and thrombosis. MF can arise following a history of polycythemia vera (PV) or essential thrombocythemia (ET), or can present de novo as primary myelofibrosis (PMF). The disease course is variable with median survival ranging from months to years. Clinical and biological features such as advanced age, leukocytosis, anemia, transfusion dependence, and elevated inflammatory markers can impact prognosis in patients with PMF. Cytogenetic abnormalities and molecular markers such as JAK2 V617F, ASXL1, and CALR mutations have also been identified as prognostic variables. Several different scoring systems have been developed based on these prognostic factors. In this review, we will discuss the clinical, biological, molecular, and cytogenetic prognostic factors that have been identified in PMF, and the current prognostic models that have been developed to guide treatment decisions.
authors.affiliation: Washington University School of Medicine, Division of Hematology, 660 S. Euclid Ave, Campus Box 8056, St. Louis, MO 63110, USA. Electronic address: azhou@dom.wustl.edu.
authors.forename: Amy
authors.initials: A
authors.lastname: Zhou
authors.affiliation: Washington University School of Medicine, Division of Hematology, 660 S. Euclid Ave, Campus Box 8125, St. Louis, MO 63110, USA. Electronic address: stoh@dom.wustl.edu.
authors.forename: Stephen T
authors.initials: ST
authors.lastname: Oh
mesh_terms: D000367:Age Factors; D000368:Aged; D037282:Calreticulin / Q000235:genetics; D006801:Humans; D053614:Janus Kinase 2 / Q000235:genetics; D008875:Middle Aged; D009154:Mutation; D011087:Polycythemia Vera / Q000175:diagnosis* / Q000235:genetics / Q000401:mortality / Q000473:pathology; D055728:Primary Myelofibrosis / Q000175:diagnosis* / Q000235:genetics / Q000401:mortality / Q000473:pathology; D011379:Prognosis; D012097:Repressor Proteins / Q000235:genetics; D012307:Risk Factors; D016019:Survival Analysis; D013920:Thrombocythemia, Essential / Q000175:diagnosis* / Q000235:genetics / Q000401:mortality / Q000473:pathology
chemical_list: C473949:ASXL1 protein, human; D037282:Calreticulin; D012097:Repressor Proteins; C507924:JAK2 protein, human; D053614:Janus Kinase 2
keywords: myelofibrosis; prognosis; survival