Suppr超能文献

自身免疫性溶血性贫血的治疗:来自墨西哥一家参考中心的真实世界数据。

Treatment of autoimmune hemolytic anemia: real world data from a reference center in Mexico.

作者信息

Jaime-Pérez José Carlos, Aguilar-Calderón Patrizia, Salazar-Cavazos Lorena, Gómez-De León Andrés, Gómez-Almaguer David

机构信息

Department of Hematology, Internal Medicine Division, Dr. Josè E. González University Hospital, School of Medicine of the Universidad Autónoma de Nuevo León, Monterrey, Mexico.

出版信息

Blood Res. 2019 Jun;54(2):131-136. doi: 10.5045/br.2019.54.2.131. Epub 2019 Jun 25.

Abstract

BACKGROUND

Warm autoimmune hemolytic anemia (w-AIHA) is an uncommon disease with heterogeneous response to treatment. Steroids are the standard treatment at diagnosis, whereas rituximab has recently been recommended as the second-line therapy of choice. Our main objective was to document the response to treatment in patients with newly diagnosed w-AIHA, including the effectiveness of low-dose rituximab as frontline treatment and for refractory disease.

METHODS

Patients with w-AIHA from 2002 to 2017 were included. Relapse-free survival (RFS), probability of maintained response (MR), and time-to-response were analyzed using the Kaplan-Meier method. Response was classified as complete, partial, and no response.

RESULTS

We included 64 adults with w-AIHA (39 women and 25 men). The median age was 37 (16-77) years. Response rates to steroids alone were 76.7%, rituximab plus steroids, 100%; and cyclophosphamide, 80%. RFS with steroids at 6, 36, and 72 months was 86.3%, 65.1%, and 59.7%, respectively. Eighteen patients received rituximab at 100 mg/wk for 4 weeks plus high-dose dexamethasone as first-line therapy, with RFS at 6, 36, and 72 months of 92.3%, 58.7% and 44.1%, respectively. Eight patients refractory to several lines of therapy were treated with low-dose rituximab, and all achieved a response (three complete response and five partial response) at a median 16 days (95% confidence interval, 14.1-17.8), with a 75% probability of MR at 103 months; the mean MR was 81.93±18 months.

CONCLUSION

Outcomes of w-AIHA treatment were considerably heterogeneous. Low rituximab doses plus high dexamethasone doses were effective for refractory disease.

摘要

背景

温抗体型自身免疫性溶血性贫血(w - AIHA)是一种罕见疾病,对治疗的反应具有异质性。类固醇是诊断时的标准治疗方法,而利妥昔单抗最近被推荐作为二线治疗的首选。我们的主要目标是记录新诊断的w - AIHA患者的治疗反应,包括低剂量利妥昔单抗作为一线治疗和用于难治性疾病的有效性。

方法

纳入2002年至2017年的w - AIHA患者。使用Kaplan - Meier方法分析无复发生存期(RFS)、维持缓解概率(MR)和缓解时间。缓解分为完全缓解、部分缓解和无缓解。

结果

我们纳入了64例成人w - AIHA患者(39名女性和25名男性)。中位年龄为37岁(16 - 77岁)。单独使用类固醇的缓解率为76.7%,利妥昔单抗加类固醇为100%;环磷酰胺为80%。使用类固醇在6个月、36个月和72个月时的RFS分别为86.3%、65.1%和59.7%。18例患者接受利妥昔单抗100mg/周,共4周加高剂量地塞米松作为一线治疗,6个月、36个月和72个月时的RFS分别为92.3%、58.7%和44.1%。8例对多线治疗难治的患者接受低剂量利妥昔单抗治疗,所有患者均在中位16天(95%置信区间,14.1 - 17.8)达到缓解(3例完全缓解和5例部分缓解),103个月时MR概率为75%;平均MR为81.93±18个月。

结论

w - AIHA治疗的结果存在很大异质性。低剂量利妥昔单抗加高剂量地塞米松对难治性疾病有效。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2e26/6614091/5c439ca11ef3/br-54-131-g001.jpg

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验