[Chronic myeloproliferative disorders. The new WHO classification].
Except for chronic myelogenous leukemia (CML), chronic myeloproliferative disorders (CMPDs) include as main subtypes polycythemia vera (PV), chronic idiopathic myelofibrosis (IMF), and essential thrombocythemia (ET). A common finding in CMPDs is a clonal evolution associated with a significantly variable course, which may be complicated by thrombocythemia, (secondary) myelofibrosis, and finally acceleration (unstable phase) that merges into blastic crisis. New therapeutic modalities (chemo- and interferon therapy, bone marrow and stem cell transplantation) which were developed in the last decade and the striking differences in survival amongst the different subtypes warrant not only an unequivocal distinction from reactive and allied disorders, but a clear-cut classification as well. For this reason, a synoptical approach is essential including clinical data and, as a major diagnostic tool, a bone marrow biopsy. This concept finds expression in the new WHO classification, which also includes as rare subtypes chronic neutrophilic leukemia, eosinophilic leukemia, chronic hypereosinophilic syndrome, and finally unclassifiable entities. Histopathology of bone marrow biopsies reveals specific findings, in particular concerning megakaryopoiesis, which are characteristic for the different subtypes. These features facilitate the still controversially discussed differentiation of thrombocythemia that is frequently present, as is the case in initial (prefibrotic) IMF from ET. Moreover, in addition to clinical findings,the associated heterogeneity of bone marrow morphology indicates a stepwise evolution of the disease process and thus exerts a significant impact on survival, i.e., in CML regarding erythropoiesis and myelofibrosis and in IMF extent of myeloid metaplasia.
Thiele
J
J
Zentrum für Pathologie der Universität zu Köln. j.thiele@uni-koeln.de
Kvasnicka
H M
HM
D001853:Bone Marrow / Q000473:pathology; D003937:Diagnosis, Differential; D006801:Humans; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000145:classification / Q000473:pathology; D009196:Myeloproliferative Disorders / Q000145:classification* / Q000473:pathology; D011087:Polycythemia Vera / Q000145:classification / Q000473:pathology; D055728:Primary Myelofibrosis / Q000145:classification / Q000473:pathology; D011379:Prognosis; D013920:Thrombocythemia, Essential / Q000145:classification / Q000473:pathology; D014944:World Health Organization
Chronische myeloproliferative Systemerkrankungen. Die neue WHO-Klassifikation.