Intravascular lymphoma (IVL) is a rare, aggressive subtype of non-Hodgkin lymphoma (NHL) characterized by the selective proliferation of neoplastic lymphoid cells within small and medium-sized blood vessels, most frequently of B-cell origin (IVLBCL). Its protean clinical presentation, lack of pathognomonic findings, and absence of tumor masses or lymphadenopathies often lead to diagnostic delays and poor outcomes. IVLBCL can manifest in classic, hemophagocytic syndrome-associated (HPS), or cutaneous variants, with extremely variable organ involvement including the central nervous system (CNS), skin, lungs, and endocrine system. Diagnosis requires histopathologic identification of neoplastic intravascular lymphoid cells via targeted or random tissue biopsies. Tumor cells are highly atypical and display a non-GCB B-cell phenotype, often expressing CD20, MUM1, BCL2, and MYC; molecularly, they frequently harbor mutations inMYD88andCD79B, defining a molecular profile shared with ABC-type DLBCL of immune-privileged sites. Therapeutic approaches are based on rituximab-containing chemotherapy regimens (R-CHOP), often supplemented with CNS-directed therapy due to the disease’s marked neurotropism. Emerging strategies include autologous stem cell transplantation (ASCT) and novel immunotherapeutic approaches, potentially exploiting the frequent expression of PD-L1 by tumor cells. A distinct but related entity, intravascular NK/T-cell lymphoma (IVNKTCL), is an exceedingly rare EBV-associated lymphoma, showing unique own histologic, immunophenotypic, and molecular features and an even poorer outcome. This review provides a comprehensive overview of the current understandings about clinicopathological, molecular, and therapeutic landscape of IVL, emphasizing the need for increased clinical awareness, standardized diagnostic protocols, and individualized treatment strategies for this aggressive yet intriguing malignancy.
血管内淋巴瘤（IVL）是一种罕见且侵袭性的非霍奇金淋巴瘤（NHL）亚型，其特征为肿瘤性淋巴细胞选择性增殖于中小血管腔内，最常见为B细胞起源（IVLBCL）。其临床表现多样、缺乏特异性征象，且不形成肿瘤包块或淋巴结肿大，常导致诊断延误和预后不良。IVLBCL可分为经典型、噬血细胞综合征相关型及皮肤型等亚型，可累及中枢神经系统、皮肤、肺和内分泌系统等多种器官，受累范围差异极大。诊断需通过靶向或随机组织活检，在组织病理学上识别血管内肿瘤性淋巴细胞。肿瘤细胞呈高度异型性，表现为非生发中心B细胞表型，常表达CD20、MUM1、BCL2和MYC；分子层面多携带MYD88和CD79B基因突变，形成与免疫豁免区ABC型弥漫大B细胞淋巴瘤相似的分子谱系。治疗方案以含利妥昔单抗的化疗方案（R-CHOP）为基础，因该病显著嗜神经特性常需联合中枢神经系统定向治疗。新兴策略包括自体干细胞移植及新型免疫疗法，后者可能利用肿瘤细胞频繁表达的PD-L1靶点。血管内NK/T细胞淋巴瘤（IVNKTCL）作为独立但相关的疾病实体，是极为罕见的EBV相关淋巴瘤，具有独特的组织学、免疫表型和分子特征，预后更差。本综述系统阐述了当前对IVL临床病理学、分子特征及治疗格局的认识，强调需提高临床警惕性、建立标准化诊断流程并制定个体化治疗策略，以应对这一兼具侵袭性与复杂性的恶性肿瘤。
Intravascular Lymphoma: A Unique Pattern Underlying a Protean Disease
肿瘤(癌症)患者之家