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Treatment Algorithm of Essential Thrombocythemia

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Pathogenesis and Treatment of Leukemia

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Abstract

Essential thrombocythemia (ET), one of the BCR-ABL-negative myeloproliferative neoplasms (MPNs), is a hematopoietic malignancy characterized by overproduction of platelets due to clonal expansion of megakaryocytes. Enhanced constitutive JAK-STAT (janus kinase 2—signal transducer and activator of transcription) signaling is central to disease pathophysiology. ET, presenting with persistent thrombocytosis, may represent a myriad of conditions and careful establishing of an accurate diagnosis is key to subsequent optimal management. This chapter reviews in detail goals of therapy in ET and treatment strategies incorporating cytoreductive, noncytoreductive, and novel therapies. Finally, approaches to specific scenarios and their management are discussed including young patients, triple-negative for classical driver mutation ET patients, pregnancy, and splanchnic vein thrombosis.

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Authors and Affiliations

  1. Department of Haematology, Guy’s and St. Thomas’ NHS Foundation Trust, London, UK

    Jennifer O’Sullivan & Claire Harrison

  2. Department of Cellular Pathology, Guys and St Thomas’ NHS Hospitals, London, UK

    Anna Green

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  1. Jennifer O’Sullivan
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Correspondence to Claire Harrison .

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  1. Department of Medicine, Queen Mary Hospital, Hongkong, Hong Kong

    Harinder Gill

  2. Department of Medicine, Queen Mary Hospital, Hongkong, Hong Kong

    Yok-Lam Kwong

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O’Sullivan, J., Green, A., Harrison, C. (2023). Treatment Algorithm of Essential Thrombocythemia. In: Gill, H., Kwong, YL. (eds) Pathogenesis and Treatment of Leukemia. Springer, Singapore. https://doi.org/10.1007/978-981-99-3810-0_38

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