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首页> 外文期刊>British Journal of Haematology >Clinical evaluation of the European LeukaemiaNet criteria for clinicohaematological response and resistance/intolerance to hydroxycarbamide in essential thrombocythaemia.
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Clinical evaluation of the European LeukaemiaNet criteria for clinicohaematological response and resistance/intolerance to hydroxycarbamide in essential thrombocythaemia.

机译：欧洲LeukaemiaNet标准对原发性血小板增多症的临床血液学反应和对羟脲的耐药性/不耐受性的临床评价。

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摘要

Standardized criteria of response to treatment and a unified definition of resistance/intolerance to hydroxycarbamide (HC) in essential thrombocythaemia (ET) have been proposed by the European LeukaemiaNet (ELN). We have retrospectively evaluated such criteria in 166 ET patients treated with HC for a median of 4·5 years. Overall, 134 patients achieved a complete clinicohaematological response (CR) and 25 a partial response. Thirty-three patients met at least one of the ELN criteria defining resistance (n = 15) or intolerance (n = 21) to HC. Fifteen cases developed anaemia with thrombocytosis, which was associated with a high incidence of myelofibrosis and death from any cause. Other definitions of resistance were less useful. Factors determining the thrombotic risk were a history of prior thrombosis and a baseline leucocyte count >10 × 10?/ l. Of note, patients achieving a CR, even if sustained during the entire follow-up, did not benefit from a lower incidence of thrombosis or an improved survival. In conclusion, most ET patients respond to HC, but the achievement of response, as defined by the ELN, does not correlate with the patients' outcome. The best discriminating ELN criterion of resistance to HC was the detection of anaemia, which also identified a subgroup of patients with poor prognosis.
机译：欧洲白血病网（ELN）提出了对治疗反应的标准化标准以及对原发性血小板增多症（ET）中对羟基脲（HC）的耐药性/不耐受性的统一定义。我们回顾性评估了166例接受HC治疗，中位4·5年的ET患者的标准。总体而言，有134例患者达到了完全的临床血液学反应（CR），有25例达到了部分反应。 33位患者至少符合ELN标准之一，该标准定义了对HC的耐药性（n = 15）或不耐受（n = 21）。 15例发展为贫血伴血小板增多症，这与骨髓纤维化的高发生率和任何原因的死亡有关。电阻的其他定义不太有用。决定血栓形成危险的因素是既往血栓形成的病史和基线白细胞计数> 10×10？/ l。值得注意的是，即使在整个随访期间持续获得CR的患者，也不会因血栓形成的发生率较低或存活率提高而受益。总之，大多数ET患者对HC有反应，但ELN定义的反应完成与患者的预后无关。 ELN对HC耐药性的最佳区分标准是贫血的检测，这也确定了预后较差的亚组患者。

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