title: Myelofibrosis: an update on current pharmacotherapy and future directions.
abstract: BACKGROUND
  Myelofibrosis (MF) is a myeloproliferative neoplasm characterized by symptoms mainly derived from anemia and splenomegaly and constitutional symptoms and associated with a median survival around 6 years. Allogeneic stem cell transplantation (allo-SCT) remains the only curative therapy of MF but is applicable to a minority of patients. Discovery of the JAK2 mutation has provided the basis for the introduction of a new class of drugs, the JAK inhibitors, in the treatment of MF.
  
  
  METHODS
  A literature review on the therapy of MF has been performed through a PubMed search, with special attention being paid to the available data on transplantation, the JAK inhibitors, and other new drugs.
  
  
  CONCLUSIONS
  Conventional therapy of MF is usually adjusted to the predominant clinical symptoms in each patient, and its impact on survival is limited. Reduced-intensity conditioning regimens have increased the number of patients eligible for allo-SCT, but this procedure is still associated with substantial morbidity and mortality. The JAK inhibitors, such as ruxolitinib, can achieve profound symptomatic relief of the splenomegaly and the constitutional symptoms. However, they often accentuate the anemia and do not reduce the JAK2 allele burden, therefore lacking the potential to modify the natural history of MF.
authors.affiliation: University of Barcelona, Hospital Clínic, Hematology Department, IDIBAPS, Villarroel 170, 08036 Barcelona, Spain. fcervan@clinic.ub.es
authors.forename: Francisco
authors.initials: F
authors.lastname: Cervantes
authors.forename: Alejandra
authors.initials: A
authors.lastname: Martinez-Trillos
mesh_terms: D000367:Age Factors; D000728:Androgens / Q000627:therapeutic use; D000740:Anemia / Q000188:drug therapy / Q000209:etiology; D006801:Humans; D007155:Immunologic Factors / Q000494:pharmacology / Q000627:therapeutic use; D053614:Janus Kinase 2 / Q000037:antagonists & inhibitors / Q000235:genetics; D009154:Mutation; D009570:Nitriles; D055728:Primary Myelofibrosis / Q000175:diagnosis / Q000235:genetics / Q000628:therapy*; D011379:Prognosis; D011720:Pyrazoles / Q000494:pharmacology / Q000627:therapeutic use; D011743:Pyrimidines; D011878:Radiotherapy; D013163:Splenomegaly / Q000188:drug therapy / Q000209:etiology; D033581:Stem Cell Transplantation / Q000379:methods*; D013792:Thalidomide / Q000031:analogs & derivatives / Q000494:pharmacology / Q000627:therapeutic use
chemical_list: D000728:Androgens; D007155:Immunologic Factors; D009570:Nitriles; D011720:Pyrazoles; D011743:Pyrimidines; D013792:Thalidomide; C540383:ruxolitinib; C467566:pomalidomide; C507924:JAK2 protein, human; D053614:Janus Kinase 2