文章：

来那度胺有效治疗老年原发性积液性淋巴瘤1例：病例报告及文献复习

Primary effusion lymphoma in an elderly patient effectively treated by lenalidomide: case report and review of literature

原文发布日期：2014-03-07

DOI: 10.1038/bcj.2014.6

类型: Review

开放获取: 是

英文摘要：

Primary effusion lymphoma (PEL) is a rare aggressive subset of non-Hodgkin B-cell lymphoma. It is caused by Kaposi sarcoma-associated herpesvirus/human herpesvirus type 8 (KSHV/HHV8). It occurs mainly, but not exclusively, in HIV-positive patients. PEL predominantly develops in serous cavities and occasionally in extracavitary regions. PEL carries a very poor prognosis with a median survival time of <6 months. Indeed, currently used treatment modalities such as CHOP chemotherapy are far from achieving complete and sustainable remission. Therefore, there is no clear standard of care established in the treatment of PEL patients, stressing the need for novel-targeted approaches. Here, we have attempted a comprehensive assessment of the treatment of PEL, discussed avant-garde therapies and updated the state of preclinical research with promising clinical applications in the field. These include inhibitors of viral replication, modulators of cell signaling and inflammation, nuclear factor kappa B (NF-κB) and histone deacetylase inhibitors, and recently the combination of arsenic trioxide and interferon-alpha. Some of these targeted therapies have not yet reached clinical studies, although others were used in a few individual case reports with low numbers of patients. We also describe the first case of a 77-year-old, HIV-negative, HHV8-positive patient diagnosed with PEL limited to the pleural and peritoneal cavities. He received lenalidomide 25 mg/day for 21 days every 28 days. Treatment was well tolerated with no side effects. He rapidly improved after 1 month of treatment and progressively achieved complete remission persistent after 18 months of therapy. We believe that this review will bridge an important gap between classical chemotherapy and modern approaches of targeted therapy. Finally, our findings warrant further evaluation of lenalidomide in future prospective clinical studies.

摘要翻译： 

原发性渗出性淋巴瘤（PEL）是一种罕见的侵袭性非霍奇金B细胞淋巴瘤亚型，由卡波西肉瘤相关疱疹病毒/人类疱疹病毒8型（KSHV/HHV8）引发。该病主要（但非绝对）发生于HIV阳性患者，通常累及浆膜腔，偶见于腔外区域。PEL预后极差，中位生存期不足6个月。事实上，当前采用的治疗方式（如CHOP方案化疗）远不能实现持续完全缓解，因此至今尚未建立明确的PEL治疗标准，这凸显了开发新型靶向疗法的迫切需求。本文系统评估了PEL的治疗现状，探讨了前沿疗法并更新了具有临床转化潜力的临床前研究进展，包括病毒复制抑制剂、细胞信号与炎症调节剂、核因子κB（NF-κB）抑制剂、组蛋白去乙酰化酶抑制剂，以及近年出现的三氧化二砷联合干扰素-α方案。部分靶向疗法虽尚未进入临床研究阶段，但已有少量个案报告。我们同时报道一例77岁HIV阴性、HHV8阳性患者的特殊病例，该患者被确诊为仅局限于胸膜腔和腹膜腔的PEL，采用来那度胺25mg/日（连续21天/28天周期）治疗方案。治疗过程耐受性良好且无副作用，用药1个月后病情迅速改善，18个月后达到持续完全缓解。我们认为本综述将填补传统化疗与现代靶向治疗之间的重要空白。最后，我们的研究结果支持在未来前瞻性临床研究中进一步评估来那度胺的疗效。

原文链接：

Primary effusion lymphoma in an elderly patient effectively treated by lenalidomide: case report and review of literature