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[阿那格雷治疗骨髓增殖性血小板增多症:6例患者的长期经验]

[Anagrelide in control of myeloproliferative thrombocythemia: long-term experiences in 6 patients].

作者信息

Burkhard R, Adam H, Widmer L, Honegger H P

机构信息

Institut für Medizinische Onkologie und Hämatologie, Stadtspital Triemli, Zürich.

出版信息

Schweiz Med Wochenschr. 1998 Nov 14;128(46):1808-12.

PMID:9857387
Abstract

Symptomatic or asymptomatic chronic elevation of platelet count can be observed in all forms of myeloproliferative disorders (MPD). Benefits and limitations of the traditional platelet-reducing agents, such as radioactive phosphorus, alkylating agents, hydroxyurea and interferon alpha, are well known and have been largely described. Anagrelide (Agrelin) is an additional newer drug with a selective platelet-lowering effect. We describe our own long-term experience in 6 patients with MPD who were treated with anagrelide as part of a compassionate-use protocol between April 1991 and August 1997. The median duration of therapy was 54 months (range 17 to 75 months), with an overall response rate of 100% (complete and partial response for at least 4 weeks). The initial median platelet count of 1211 x 10(9)/l (range 847 to 2050 x 10(9)/l) was reduced rapidly and lastingly to 570 x 10(9)/l (range 216 to 667 x 10(9)/l) at the time of the last control. Under treatment with anagrelide 4 of the 6 patients showed a reduction of disease-associated symptoms or complications. Adverse reactions were generally mild and transitory, and no interruption or cessation of therapy was required. Development of drug resistance or late adverse events were not observed. Treatment with anagrelide is effective, safe and in our opinion easy to administer. It also appears to be suitable for long-term administration.

摘要

在所有类型的骨髓增殖性疾病(MPD)中,均可观察到有症状或无症状的血小板计数慢性升高。传统的血小板减少剂,如放射性磷、烷化剂、羟基脲和α干扰素,其利弊已广为人知且多有描述。阿那格雷(Agrelin)是另一种具有选择性降低血小板作用的新药。我们描述了1991年4月至1997年8月期间,6例MPD患者作为同情用药方案的一部分接受阿那格雷治疗的长期经验。治疗的中位持续时间为54个月(范围17至75个月),总体缓解率为100%(完全缓解和部分缓解至少持续4周)。初始血小板计数中位数为1211×10⁹/L(范围847至2050×10⁹/L),在最后一次复查时迅速且持久地降至570×10⁹/L(范围216至667×10⁹/L)。在阿那格雷治疗期间,6例患者中有4例疾病相关症状或并发症有所减轻。不良反应一般轻微且短暂,无需中断或停止治疗。未观察到耐药性的出现或晚期不良事件。阿那格雷治疗有效、安全,且我们认为易于给药。它似乎也适合长期给药。

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