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Latest progress in the diagnosis of early/prefibrotic primary myelofibrosis and its comparison with essential thrombocythemia |
Received:October 22, 2018 |
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DOI:10.11915/j.issn.1671-5403.2019.03.048 |
Key words:myeloproliferative neoplasms thrombocythemia early/prefibrotic primary myelofibrosis diagnosis prognosis |
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Abstract: |
Myeloid neoplasms and acute leukemia are categorized as early/prefibrotic primary myelofibrosis (Pre-PMF) and overt primary myelofibrosis (Overt PMF) in the revised World Health Organization classification in 2016. Thrombocytosis is a common manifestation in the patients with Pre-PMF and is similar to the essential thrombocythemia (ET) at onset. However, Pre-PMF and ET differ in survival rate, leukemic evolution and rates of progression to Overt PMF, and accurate differentiation is of vital importance. The diagnosis of Pre-PMF is mainly based on histopathological features of bone marrow biopsy, fibrosis grade and clinical parameters(leukocytosis, anemia, elevated lactate dehydrogenase and splenomegaly). The current article summarizes the latest advances in Pre-PMF diagnosis and compares its clinical features,morphological changes,molecular genetics and prognosis with those of ET. |
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