Intravascular Large B-cell lymphoma: A case series and review of literatures.
Intravascular Large B-cell lymphoma: A case series and review of literatures.
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DOI：
10.1016/j.bj.2020.04.005
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发表时间：
2021-08
影响因子：
5.5
通讯作者：
Kuo MC
中科院分区：
文献类型：
--
作者：
Ong YC;Kao HW;Chuang WY;Hung YS;Lin TL;Chang H;Kuo MC
血管内大B细胞淋巴瘤（IVLBCL）是非霍奇金淋巴瘤的一种罕见亚型，临床表现少见，预后差。本研究的目的是在台湾的一个单一的机构报告IVLBCL的临床特征和结果。回顾性分析了2006年6月至2018年1月诊断的10例IVLBCL患者。中位年龄为61岁（范围39-88岁）。最常见的表现是发热（90%）、血细胞减少（90%）和意识模糊（50%）。所有患者的中位无进展生存期（PFS）和总生存期（OS）分别为12.6（95%置信区间[CI] 0.0-76.1）和18.8（95% CI 0-59.3）个月。6例患者接受利妥昔单抗联合化疗，另1例患者仅接受化疗。7例患者中有6例（85.7%）在化疗后获得完全缓解。6例完成治疗的患者未达到中位PFS和OS。3年PFS和OS率分别为80%和75%。我们的研究表明，患者在利妥昔单抗为基础的化疗后可能获得持久缓解。IVLBCL患者如能早期诊断，及时治疗，预后可进一步改善。
Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of non-Hodgkin lymphoma with uncommon clinical presentations and poor prognosis. The purpose of this study is to report the clinical features and outcome of IVLBCL in a single institution of Taiwan. Ten patients with IVLBCL diagnosed from June 2006 to January 2018 were retrospectively reviewed. The median age was 61 (range 39–88) years. The most common presentation was fever (90%), cytopenia (90%), and confusion (50%). For all patients, the median progression free survival (PFS) and overall survival (OS) were 12.6 (95% confidence interval [CI] 0.0–76.1) and 18.8 (95% CI 0–59.3) months, respectively. Six patients received rituximab combined chemotherapy, and the other one patient was treated with chemotherapy alone. Six of seven (85.7%) patients achieved complete response after chemotherapy. The median PFS and OS for six patients who completed treatment were not reached. Three-year PFS and OS rates were 80% and 75%, respectively. Our study showed that patients might achieve durable remission after rituximab-based chemotherapy. The outcome of IVLBCL patients may further improve if early diagnosis and prompt treatment were made.
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