TY - JOUR
T1 - Prognostication in MF
T2 - From CBC to cytogenetics to molecular markers
AU - Zhou, Amy
AU - Oh, Stephen T.
N1 - Publisher Copyright:
© 2014 Elsevier Ltd. All rights reserved.
PY - 2014
Y1 - 2014
N2 - Myelofibrosis (MF) is a clonal stem cell disorder characterized by ineffective erythropoiesis and extramedullary hematopoiesis leading to progressive bone marrow failure, severe anemia, constitutionalsymptoms, hepatosplenomegaly, and thrombosis.MF can arise following a history of polycythemia vera (PV) or essential thrombocythemia (ET), or canpresent de novo as primarymyelofibrosis(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.
AB - Myelofibrosis (MF) is a clonal stem cell disorder characterized by ineffective erythropoiesis and extramedullary hematopoiesis leading to progressive bone marrow failure, severe anemia, constitutionalsymptoms, hepatosplenomegaly, and thrombosis.MF can arise following a history of polycythemia vera (PV) or essential thrombocythemia (ET), or canpresent de novo as primarymyelofibrosis(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.
KW - Myelofibrosis
KW - Prognosis
KW - Survival
UR - http://www.scopus.com/inward/record.url?scp=84927971002&partnerID=8YFLogxK
U2 - 10.1016/j.beha.2014.07.008
DO - 10.1016/j.beha.2014.07.008
M3 - Review article
C2 - 25189726
AN - SCOPUS:84927971002
SN - 1521-6926
VL - 27
SP - 155
EP - 164
JO - Best Practice and Research: Clinical Haematology
JF - Best Practice and Research: Clinical Haematology
IS - 2
ER -