title: [Myeloproliferative neoplasms: histopathological and molecular pathological diagnosis].
abstract: Myeloproliferative neoplasms (chronic myeloproliferative disorders according to former nomenclature) comprise chronic myeloid leukemia, polycythemia vera, essential thrombocythemia, primary myelofibrosis, chronic eosinophilic leukemia, chronic neutrophilic leukemia and systemic mastocytosis. All disorders have excessive proliferation of one or more hematopoietic lineages in common and progress with different probability to blast crisis or fibrosis. A further common feature is provided by the activating mutation of tyrosin kinases and associated pathways of signal transduction (BCR-ABL, JAK2(V617F), MPL(W515L/K), KIT(D816V) and FIP1L1-PDGFRA) causative for the abnormal proliferation. With regard to diagnosis and therapy these mutations are of utmost importance because they enable the exclusion of reactive processes, contribute with varying specificity to subtyping of MPN and are at least partly sensitive to targeted therapy. The molecular mechanisms of blastic and fibrotic progression are not yet understood.
authors.affiliation: Institut für Pathologie, Medizinische Hochschule Hannover, Carl-Neuberg-Str 1, 30625 Hannover, Deutschland.
authors.forename: K
authors.initials: K
authors.lastname: Hussein
authors.forename: G
authors.initials: G
authors.lastname: Büsche
authors.forename: J
authors.initials: J
authors.lastname: Schlue
authors.forename: U
authors.initials: U
authors.lastname: Lehmann
authors.forename: H
authors.initials: H
authors.lastname: Kreipe
mesh_terms: D014408:Biomarkers, Tumor / Q000235:genetics; D001752:Blast Crisis / Q000235:genetics / Q000473:pathology; D001853:Bone Marrow / Q000473:pathology*; D001856:Bone Marrow Examination / Q000379:methods*; D002471:Cell Transformation, Neoplastic / Q000235:genetics / Q000473:pathology; D002869:Chromosome Aberrations; D004252:DNA Mutational Analysis; D005819:Genetic Markers / Q000235:genetics; D006801:Humans; D007150:Immunohistochemistry; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000235:genetics* / Q000473:pathology*; D025202:Molecular Diagnostic Techniques; D054437:Myelodysplastic-Myeloproliferative Diseases / Q000235:genetics* / Q000473:pathology*; D009367:Neoplasm Staging
chemical_list: D014408:Biomarkers, Tumor; D005819:Genetic Markers