Myeloproliferative neoplasms 2012: the John M. Bennett 80th birthday anniversary lecture.
Polycythemia vera, essential thrombocythemia and primary myelofibrosis constitute the BCR-ABL1-negative myeloproliferative neoplasms. In this communication, I will provide an overview on their histopathology, cytogenetic findings and associated mutations, as well as summarize recent advances that have changed our approach to their diagnosis and treatment. Also included in the current review are (i) indications for ordering JAK2 or MPL mutation analysis and result interpretation, (ii) new international prognostic scoring systems, and (iii) risk-adapted therapy including the therapeutic role of immunomodulatory drugs and JAK inhibitors.
Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN 55905, USA. tefferi.ayalew@mayo.edu
Ayalew
A
Tefferi
D000970:Antineoplastic Agents / Q000627:therapeutic use; D020732:Cytogenetic Analysis; D016044:Fusion Proteins, bcr-abl / Q000235:genetics; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D053614:Janus Kinase 2 / Q000235:genetics; D009154:Mutation; D011087:Polycythemia Vera / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D055728:Primary Myelofibrosis / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use; D053628:Receptors, Thrombopoietin / Q000235:genetics; D013920:Thrombocythemia, Essential / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics / Q000473:pathology
D000970:Antineoplastic Agents; D007155:Immunologic Factors; D047428:Protein Kinase Inhibitors; D053628:Receptors, Thrombopoietin; C082218:MPL protein, human; D016044:Fusion Proteins, bcr-abl; C507924:JAK2 protein, human; D053614:Janus Kinase 2