Current progress and future perspectives of research on intravascular large B-cell lymphoma.
Current progress and future perspectives of research on intravascular large B-cell lymphoma.
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DOI：
10.1111/cas.15091
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发表时间：
2021-10
期刊：
影响因子：
5.7
通讯作者：
Kiyoi H
中科院分区：
文献类型：
--
作者：
Shimada K;Kiyoi H
血管内大B细胞淋巴瘤是一种罕见的大B细胞疾病，其特征是选择性生长在全身器官的小血管管腔内。自从1959年首次报道以来，从活检标本中获得足够的肿瘤细胞的困难阻碍了对其潜在生物学的阐明。利用异种移植模型和无浆细胞DNA的最新进展发现了类似于激活的B细胞型弥漫性大B细胞淋巴瘤的遗传特征，包括MYD88和CD79B突变以及免疫检查点相关基因如PD-L1和PD-L2的频繁变化。鉴于利妥昔单抗时代临床结果的改善和继发性中枢神经系统(CNS)受累的风险增加，R-CHOP联合大剂量甲氨蝶呤和鞘内化疗作为一种以CNS为导向的治疗方法进行了2期试验。这项试验，即PRIMUR-IVL研究，显示了良好的无进展存活率和较低的继发性中枢神经系统受累累积发生率。这项试验的长期随访仍在进行中。仍需进一步了解该病的病理生理学机制和改善临床结果。血管内大B细胞淋巴瘤是一种罕见的结外大B细胞淋巴瘤。IVLBCL的临床和翻译研究的最新进展使我们能够了解该病的生物学特征，并应用积极的治疗方法。有必要进行进一步的研究，以加深我们对这种疾病的了解，并建立最佳的治疗方法。
Intravascular large B‐cell lymphoma is a rare disease of the large B cells characterized by selective growth in the lumina of small vessels in systemic organs. Since first reported in 1959, the difficulty of obtaining sufficient tumor cells from biopsy specimens has hampered the elucidation of its underlying biology. Recent progress using xenograft models and plasma cell‐free DNA has uncovered genetic features that are similar to those of activated B‐cell type diffuse large B‐cell lymphoma, including MYD88 and CD79B mutations and frequent alterations in immune check point‐related genes such as PD‐L1 and PD‐L2. Given the improvement in clinical outcomes and a higher risk of secondary central nervous system (CNS) involvement in the rituximab era, a phase 2 trial of R‐CHOP combined with high‐dose methotrexate and intrathecal chemotherapy as a CNS‐oriented therapy has been conducted. This trial, the PRIMEUR‐IVL study, has displayed good progression‐free survival and a low cumulative incidence of secondary CNS involvement. Long‐term follow‐up within this trial is still ongoing. Further understanding of the pathophysiology of the disease and improvements in clinical outcomes are still needed. Intravascular large B‐cell lymphoma is a rare type of extranodal large B‐cell lymphoma. Recent progress on clinical and translational research on IVLBCL allows us to understand the biological features of the disease and to apply an active treatment. Further investigations are warranted to deepen our understanding of the disease and to establish optimal treatments.
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