bcr/abl-Negative, Classic Myeloproliferative Disorders: Diagnosis and Treatment

Tefferi, Ayalew; Bardui, Tiziano
September 2005
Mayo Clinic Proceedings;Sep2005, Vol. 80 Issue 9, p1220
Academic Journal
Essential thrombocythemia, polycythemia vera, and myelofibrosis with myeloid metaplasia constitute the ‘classic’ bcr/abl-negative myeloproliferative disorders (MPDs). Each of these MPDs represents a stem cell-derived clonal myeloproliferation with the respective features of thrombocytosis, erythrocytosis, and bone marrow fibrosis. Unlike with cases of chronic myeloid leukemia, in which the bcr/abl mutation is invariably detected, current diagnosis of essential thrombocythemia, polycythemia vera, and myelofibrosis with myeloid metaplasia is based on a consensus-driven set of clinical and laboratory criteria that have undergone substantial modification in recent times. The recent discovery of a recurrent activating Janus tyrosine kinase (JAK2) mutation (JAK2V617F) in all 3 classic MPDs offers another opportunity for refining current diagnoses and disease classifications. In this article, we outline contemporary diagnostic algorithms for each of these disorders and provide an evidence-based approach to management.


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