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Essential thrombocythemia (ET) is a myeloproliferative neoplasm that overproduces platelets and is associated with life-threatening thrombosis. Medical cytoreduction either with hydroxyurea (HU) or anagrelide (AG) is widely used, but drug intolerance or resistance are major concerns. Low-dose combination of HU and AG as an alternative strategy has been explored in various studies.

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Article Synopsis
  • A 62-year-old woman with essential thrombocythemia developed rapid kidney dysfunction, prompting her referral to a hospital for further investigation.
  • Tests indicated some minor issues, such as elevated urinary β2-microglobulin, but a renal biopsy revealed acute tubular necrosis without significant glomerular damage.
  • Her condition improved after switching from anagrelide to hydroxyurea, highlighting the need to consider anagrelide as a potential cause of kidney issues in patients taking it; thus, thorough medical history and biopsy are essential in these evaluations.
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Essential thrombocythaemia (ET) is a myeloproliferative neoplasm characterized by an increased risk of vascular complications and a tendency to progress to myelofibrosis and acute leukaemia. ET patients have traditionally been stratified into two thrombosis risk categories based on age older than 60 years and a history of thrombosis. More recently, the revised IPSET-thrombosis scoring system, which accounts for the increased risk linked to the JAK2 mutation, has been incorporated into most expert recommendations.

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Unlabelled: Anagrelide is a medication primarily used to manage thrombocytosis, an abnormal increase in platelet levels in the blood. It is often prescribed for patients with myeloproliferative disorders, such as essential thrombocythaemia (ET). Given the heightened susceptibility to thromboembolism associated with this condition, the primary emphasis in treatment revolves around reducing the risk of thrombotic events through the administration of cytotoxic agents.

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Article Synopsis
  • Interferon-based therapies like ropeginterferon alfa-2b are showing promise as potential treatments for essential thrombocythemia (ET), which current therapies don't modify or improve disease progression.
  • Ropeginterferon alfa-2b has been effective in treating polycythemia vera (PV) and could similarly benefit ET patients who can't tolerate or are resistant to existing treatments.
  • The ongoing ROP-ET trial aims to evaluate its effectiveness over three years, focusing on patient responses, quality of life, and safety, providing crucial data for treatment options in this underserved group.
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