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Abstract
Essential thrombocythemia is a clonal myeloproliferative neoplasm characterized by an elevated platelet count, the potential for both microvascular and macrovascular sequelae, and a risk for transformation to myelofibrosis or acute myeloid leukemia. A systematic and detailed initial analysis is essential for accurate diagnosis of essential thrombocythemia, as many etiologies are reactive and benign. Once a diagnosis has been made, risk stratification and symptom assessment are vital to guide the subsequent therapy. Treatment may be required in high-risk disease, such as in cases of advanced age or prior thrombotic events. Systemic therapy is aimed at reducing the thrombotic risk and includes daily low dose aspirin and in some patients, cytoreductive therapy. Currently, the first line cytoreductive therapy includes hydroxyurea or pegylated interferon, with a phase III clinical trial underway comparing these two important agents. Anagrelide and clinical trials are reserved for refractory or intolerant patients. Looking to the future, new therapies including Janus kinase 2 (JAK2) and telomerase inhibitors are promising and may become valuable to the treatment armamentarium for those afflicted with essential thrombocythemia.
Highlights
Thrombocythemia, or elevation in platelet count (i.e. greater than 450 × 10 (9)/L), is a common observation for internists and hematologists alike
Essential thrombocythemia (ET), one of the myeloproliferative neoplasms (MPNs), is an aberration within the bone marrow and its microenvironment leading to clonal proliferation of the megakaryocytic lineage within the marrow and, to peripheral blood thrombocythemia
We discuss the diagnostic strategy of thrombocytosis with particular attention paid to essential thrombocythemia
Summary
Thrombocythemia, or elevation in platelet count (i.e. greater than 450 × 10 (9)/L), is a common observation for internists and hematologists alike. ET is associated with thrombotic and hemorrhagic complications and requires systemic medical therapy in high-risk patients. The duration of therapy is typically lifelong, with the goal of treatment being hemorrhagic and thrombotic risk reduction, as well as retardation of disease progression For those who are intolerant to or progressed on all approved agents, clinical trials should be considered. The development of increased constitutional symptoms such as progressive splenomegaly, fever, weight loss, early satiety, and bone pain in conjunction with a trend towards either new cytopenia or increased rate of proliferative disease increases clinical suspicion of a post ETmyelofibrosis Those with new blasts on peripheral smear and/or marrow and new cytogenetic complexity should be evaluated for MPN blast phase or AML28
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