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Journal of Zhejiang University SCIENCE B
ISSN 1673-1581(Print), 1862-1783(Online), Monthly
2025 Vol.26 No.5 P.493-502
Unmet needs of patients with intravascular large B-cell lymphoma: three case reports and a literature review
Abstract: Intravascular large B-cell lymphoma (IVLBCL), a rare subtype of non-Hodgkin lymphoma, is classified as an independent subtype of extranodal diffuse large B-cell lymphoma (DLBCL) in the 2008 World Health Organization (WHO) Classification (Turner et al., 2010). The 5th edition of the World Health Organization (WHO 2022) classification of hematolymphoid tumors retains this subtype (Alaggio et al., 2022). IVLBCL, which is characterized by neoplastic lymphocyte proliferation within the lumen of small blood vessels, tends to invade organs, such as the nervous system, skin, bone marrow (BM), and lung (D'Angelo et al., 2019; Satoh et al., 2019; Vsquez et al., 2019; Fukami et al., 2020).
Key words: Intravascular large B-cell lymphoma; Clinicopathologic features; Molecular genetic characteristics; Treatment strategies
1浙江大学医学院附属第二医院血液内科，中国杭州市，310009
2浙江大学医学院附属第二医院病理科，中国杭州市，310009
3浙江大学医学院附属第二医院神经外科，中国杭州市，310009
摘要：血管内大B细胞淋巴瘤（IVLBCL）是一种具有高度侵袭性的恶性非霍奇金淋巴瘤，作为结外弥漫大B细胞淋巴瘤（DLBCL）的一种罕见亚型，其特征是小血管腔内的淋巴细胞增殖。IVLBCL早期诊断困难，预后极差。本文介绍了3例IVLBCL患者，分别描述了患者的临床特征和病理特征。所述患者均接受了免疫化疗联合大剂量甲氨蝶呤和布鲁顿酪氨酸激酶抑制剂的综合方案，但疗效有限，仅一名患者获得了良好和持久的反应。本研究回顾了相关文献并进行总结，以进一步识别IVLBCL早期诊断的临床病理特征，并基于分子遗传特征和临床亚型对IVLBCL进行了精确的预后分层，讨论了联合靶向免疫药物的治疗新策略。目前临床上IVLBCL患者的诊断及治疗需求远未被满足，因此我们需要开展更多研究，增加该疾病早期诊断和治疗的可能性，以提高患者生存率。
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DOI:
10.1631/jzus.B2300941
CLC number:
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On-line Access:
2025-05-28
Received:
2023-12-27
Revision Accepted:
2024-06-15
Crosschecked:
2025-05-29