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Hydroxyurea-associated platelet count oscillations in polycythemia vera: a report of four new cases and a review.

机译:真性红细胞增多症中与羟基脲相关的血小板计数振荡:四例新病例的报告和一篇综述。

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摘要

Cyclic oscillations in the peripheral blood platelet count were recently described in two patients with polycythemia vera (PV) receiving therapy with hydroxyurea (HU). This phenomenon can make proper HU dosing very challenging and may be especially problematic in PV patients who are at risk for thrombohemorrhagic complications. In this report, we describe four new cases of HU-associated platelet oscillations in patients with PV and extend our observations on one of the two previously reported cases. We also review cyclic thrombocytosis within the broader context of periodic hematopoiesis. A thrombopoietin-mediated negative feedback loop is central to most models of periodic thrombopoiesis, but this is probably an oversimplification. It is possible that HU destabilizes the delicate balance of thrombopoietin/c-Mpl signaling in megakaryocytic lineage hematopoiesis that is already markedly altered in PV; this hypothesis remains speculative. For patients who develop oscillatory variation in their platelet counts associated with HU use, keeping the HU dose constant may result in damping or termination of the cycles. However, this strategy is not always successful.
机译:最近在两名接受羟基脲(HU)治疗的真性红细胞增多症(PV)患者中描述了外周血血小板计数的周期性波动。这种现象会使正确的HU给药变得非常困难,对于有血栓出血性并发症风险的PV患者可能尤其成问题。在本报告中,我们描述了PV患者中与HU相关的血小板振荡的四例新病例,并将我们的观察结果扩展到先前报道的两个病例之一。我们还将在定期造血的更广泛的背景下审查循环血小板增多。血小板生成素介导的负反馈回路是大多数周期性血小板生成模型的核心,但这可能过于简化。 HU可能破坏巨核细胞谱系造血功能中血小板生成素/ c-Mpl信号转导的微妙平衡,而PV已经明显改变。这个假设仍然是推测性的。对于与HU使用相关的血小板计数出现振荡变化的患者,保持HU剂量恒定可能会导致周期减缓或终止。但是,这种策略并不总是成功的。

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