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脾切除术治疗免疫性血小板减少症:虽已减少但并未消失。

Splenectomy for immune thrombocytopenia: down but not out.

机构信息

Division of Hematology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD.

Division of Hematology and Thromboembolism, Michael G. DeGroote School of Medicine, Department of Medicine, McMaster University, Hamilton, ON, Canada; and.

出版信息

Blood. 2018 Mar 15;131(11):1172-1182. doi: 10.1182/blood-2017-09-742353. Epub 2018 Jan 2.

DOI:10.1182/blood-2017-09-742353
PMID:29295846
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5855018/
Abstract

Splenectomy is an effective therapy for steroid-refractory or dependent immune thrombocytopenia (ITP). With the advent of medical alternatives such as rituximab and thrombopoietin receptor antagonists, the use of splenectomy has declined and is generally reserved for patients that fail multiple medical therapies. Splenectomy removes the primary site of platelet clearance and autoantibody production and offers the highest rate of durable response (50% to 70%) compared with other ITP therapies. However, there are no reliable predictors of splenectomy response, and long-term risks of infection and cardiovascular complications must be considered. Because the long-term efficacy of different second-line medical therapies for ITP have not been directly compared, treatment decisions must be made without supportive evidence. Splenectomy continues to be a reasonable treatment option for many patients, including those with an active lifestyle who desire freedom from medication and monitoring, and patients with fulminant ITP that does not respond well to medical therapy. We try to avoid splenectomy within the first 12 months after ITP diagnosis for most patients to allow for spontaneous or therapy-induced remissions, particularly in older patients who have increased surgical morbidity and lower rates of response, and in young children. Treatment decisions must be individualized based on patients' comorbidities, lifestyles, and preferences. Future research should focus on comparing long-term outcomes of patients treated with different second-line therapies and on developing personalized medicine approaches to identify subsets of patients most likely to respond to splenectomy or other therapeutic approaches.

摘要

脾切除术是治疗激素难治性或依赖型免疫性血小板减少症(ITP)的有效方法。随着利妥昔单抗和血小板生成素受体拮抗剂等医学替代方法的出现,脾切除术的应用已经减少,通常仅用于多次医学治疗失败的患者。脾切除术切除了血小板清除和自身抗体产生的主要部位,与其他 ITP 治疗方法相比,提供了最高的持久反应率(50%至 70%)。然而,目前没有可靠的脾切除术反应预测指标,并且必须考虑长期感染和心血管并发症的风险。由于不同二线医学治疗 ITP 的长期疗效尚未直接比较,因此必须在没有支持证据的情况下做出治疗决策。对于许多患者,包括那些生活活跃、希望摆脱药物和监测的患者,以及对药物治疗反应不佳的暴发性 ITP 患者,脾切除术仍然是一种合理的治疗选择。我们试图避免在 ITP 诊断后的前 12 个月内进行脾切除术,以允许自发性或治疗诱导的缓解,特别是在手术发病率较高、反应率较低的老年患者和年幼的儿童中。治疗决策必须根据患者的合并症、生活方式和偏好进行个体化。未来的研究应侧重于比较不同二线治疗患者的长期结局,并开发个性化医学方法来确定最有可能对脾切除术或其他治疗方法有反应的患者亚组。

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本文引用的文献

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Thrombocytopenia in pregnancy.妊娠期血小板减少症
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Infections in non-splenectomized persistent or chronic primary immune thrombocytopenia adults: risk factors and vaccination effect.非脾切除成人持续性或慢性原发性免疫性血小板减少症的感染:危险因素和疫苗接种效果。
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