[Chronic myeloid neoplasms. Diagnostic criteria and current  therapeutic concepts].
Myeloproliferative neoplasms (MPNs) and related chronic disorders constitute a subgroup of myeloid malignancies which are defined according to clinical, morphological and molecular features by the actual World Health Organization classification of tumors of the haematopietic system. Screening procedures for a BCR-ABL fusion gene, JAK2, thrombopoietin receptor and KIT mutations are formally included in the diagnostic approach. Myelodysplastic/MPN overlap syndromes include rare entities such as refractory anemia with ringed sideroblasts characterized by a high proportion of JAK2V617F mutated cases. The paradigm of targeted treatment of chronic myeloid leukemia with imatinib has now been extended to eosinophilia-associated myeloid neoplasms with PDGFRA, PDGFRB or FGFR1 gene mutations. Pegylated interferon-alpha has convincingly been proved to reduce the JAK2 allele burden. JAK2 inhibitor drugs are currently being tested in clinical trials. The development of pathogenesis-targeted diagnostic and therapeutic approaches to the various MPNs will continue in the future.
Schmitt-Graeff
A H
AH
Institut für Pathologie, Universitätsklinikum Freiburg, Breisacherstrasse 115a, Freiburg. annette.schmitt-graeff@uniklinik-freiburg.de
D000483:Alleles; D000970:Antineoplastic Agents / Q000009:adverse effects / Q000627:therapeutic use*; D014408:Biomarkers, Tumor / Q000235:genetics; D001853:Bone Marrow / Q000473:pathology; D004252:DNA Mutational Analysis; D003937:Diagnosis, Differential; D006801:Humans; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000188:drug therapy* / Q000235:genetics / Q000473:pathology*; D015477:Leukemia, Myelomonocytic, Chronic / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D025202:Molecular Diagnostic Techniques; D009196:Myeloproliferative Disorders / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D011087:Polycythemia Vera / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D055728:Primary Myelofibrosis / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D011379:Prognosis; D011505:Protein-Tyrosine Kinases / Q000037:antagonists & inhibitors; D015398:Signal Transduction / Q000187:drug effects / Q000235:genetics
D000970:Antineoplastic Agents; D014408:Biomarkers, Tumor; D011505:Protein-Tyrosine Kinases