Clinicopathological criteria for differential diagnosis of thrombocythemias in various myeloproliferative disorders.

Thrombocythemias with the presenting or developing complications of thromboembolic and hemorrhagic episodes may be encountered at strikingly different incidences in each subtype of chronic myeloproliferative disorders. A critical reappraisal of the Polycythemia Vera Study Group (PVSG) criteria for essential thrombocythemia (ET) reveals that differentiation is explicitly focused on the exclusion of chronic myeloid leukemia and polycythemia vera (PV), but not on prodromal stages of chronic idiopathic myelofibrosis (CIMF) or latent (initial) PV. Consequently, it may be assumed that most series of patients with so-called ET include a considerable fraction of patients with the latter entities. The diagnostic impact of bone marrow (BM) histopathology was recognized by the World Health Organization classification, which emphasizes for the first time positive criteria for ET. The need of a more accurate ET diagnosis is obvious, in particular regarding therapeutic strategies and outcome (i.e., progression into myelofibrosis and blastic crisis). Conversely, early CIMF with accompanying thrombocythemia mimicking (true) ET is characterized by a higher rate of evolution into myelofibrosis and fatal complications. A scrutinized discrimination of thrombocythemias resulting in a clear-cut diagnosis of true versus false ET is warranted by a professional evaluation of BM biopsies in ongoing and prospective clinical trials.