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达那唑挽救治疗对激素和利妥昔单抗治疗无效或复发的成人免疫性血小板减少症。

Dapsone salvage therapy for adult patients with immune thrombocytopenia relapsed or refractory to steroid and rituximab.

机构信息

Clinica Ematologica, Centro Trapianti e Terapie Cellulari Carlo Melzi DISM, Azienda Ospedaliera Universitaria S. Maria Misericordia, Udine, Italy.

出版信息

Am J Hematol. 2012 Mar;87(3):321-3. doi: 10.1002/ajh.22266. Epub 2011 Dec 21.

Abstract

Dapsone is an antibacterial sulfonamide with anti-inflammatory property, which showed therapeutic activity in patients with immune thrombocytopenia (ITP); the activity in patients who showed refractoriness to rituximab is unknown. We evaluated the effect of dapsone in 20 consecutive adult patients, median age 51 years, with primary ITP previously treated at least with steroids and rituximab. Median baseline platelet count was 19 × 10⁹/L, and the median interval between diagnosis of ITP and dapsone therapy was 46 months. Response (platelet count ≥ 30 × 10⁹/L) and complete response (CR; platelet count ≥ 100 × 10⁹/L) were 55 and 20%, respectively; median time to response (TTR) was 1 month. All responders were able to interrupt any other specific anti-ITP treatment. The median duration of dapsone therapy in responders and the median response duration were 31 and 42 months, respectively. None of responders lost response during treatment. One patient in CR interrupted dapsone after 9 months and still maintained the response after 48 months. None of the patients interrupted the treatment for toxicity. All the patients were screened for normal glucose-6-phosphate-dehydrogenase (G6PD); two patients showed mild increase of methemoglobin (MHb). These results highlight the therapeutic activity and good safety profile of dapsone in patients with ITP who previously failed rituximab treatment.

摘要

氨苯砜是一种具有抗炎特性的抗菌磺胺类药物,在免疫性血小板减少症(ITP)患者中显示出治疗活性;但其在对利妥昔单抗治疗无反应的患者中的活性尚不清楚。我们评估了氨苯砜在 20 例连续的成年 ITP 患者中的疗效,这些患者的中位年龄为 51 岁,既往至少接受过皮质类固醇和利妥昔单抗治疗。中位基线血小板计数为 19×10⁹/L,ITP 诊断与氨苯砜治疗之间的中位间隔为 46 个月。反应(血小板计数≥30×10⁹/L)和完全反应(CR;血小板计数≥100×10⁹/L)分别为 55%和 20%;中位反应时间(TTR)为 1 个月。所有的反应者均能中断任何其他特定的抗 ITP 治疗。反应者的氨苯砜治疗中位持续时间和中位反应持续时间分别为 31 个月和 42 个月。所有反应者在治疗期间均未失去反应。1 例 CR 患者在 9 个月后中断氨苯砜治疗,在 48 个月后仍维持反应。无患者因毒性而中断治疗。所有患者均接受了正常葡萄糖-6-磷酸脱氢酶(G6PD)筛查;两名患者出现轻微高铁血红蛋白(MHb)升高。这些结果突出了氨苯砜在既往利妥昔单抗治疗失败的 ITP 患者中的治疗活性和良好的安全性。

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