Prodromal myeloproliferative neoplasms: the 2008 WHO classification.
The concept of prodromal chronic myeloproliferative neoplasms has been endorsed by the WHO classification implicating a stepwise evolution of disease. Histology of the bone marrow (BM) and borderline to mildly expressed clinical features play a pivotal role for diagnosing prefibrotic-early primary myelofibrosis. By lowering the platelet count for essential thrombocythemia and regarding BM morphology, early manifestations are tackled. Pre-polycythemic stages of polycythemia vera with a low hemoglobin level at onset are diagnosed by positive JAK2V617F mutation status, a low erythropoietin value, and characteristic BM features. The revised WHO classification incorporates hematological, morphological, and molecular-genetic parameters to generate a consensus-based working diagnosis.
Kvasnicka
Hans Michael
HM
Institute of Pathology, University of Cologne, Kerpener Strasse 62, Cologne, Germany. hm.kvasnicka@uni-koeln.de
Thiele
Juergen
J
D001856:Bone Marrow Examination; D018450:Disease Progression; D004911:Erythrocyte Volume; D004921:Erythropoietin / Q000097:blood; D005260:Female; D006801:Humans; D053614:Janus Kinase 2 / Q000235:genetics; D008297:Male; D009154:Mutation; D010976:Platelet Count; D011087:Polycythemia Vera / Q000175:diagnosis / Q000473:pathology*; D055728:Primary Myelofibrosis / Q000175:diagnosis / Q000473:pathology*; D012306:Risk; D013920:Thrombocythemia, Essential / Q000175:diagnosis / Q000473:pathology*; D014944:World Health Organization
D004921:Erythropoietin; C507924:JAK2 protein, human; D053614:Janus Kinase 2