Department of Medicine, Division of Hematologic Malignancies and Cellular Therapy, Duke University, Durham, NC, USA.
Department of Hematology, The National Hospital Organization Hiroshima-Nishi Medical Center, Hiroshima-ken, Japan.
Nature. 2018 Aug;560(7716):55-60. doi: 10.1038/s41586-018-0342-5. Epub 2018 Jul 18.
Acute lymphoblastic leukaemia (ALL) has a marked propensity to metastasize to the central nervous system (CNS). In contrast to brain metastases from solid tumours, metastases of ALL seldom involve the parenchyma but are isolated to the leptomeninges, which is an infrequent site for carcinomatous invasion. Although metastasis to the CNS occurs across all subtypes of ALL, a unifying mechanism for invasion has not yet been determined. Here we show that ALL cells in the circulation are unable to breach the blood-brain barrier in mice; instead, they migrate into the CNS along vessels that pass directly between vertebral or calvarial bone marrow and the subarachnoid space. The basement membrane of these bridging vessels is enriched in laminin, which is known to coordinate pathfinding of neuronal progenitor cells in the CNS. The laminin receptor α6 integrin is expressed in most cases of ALL. We found that α6 integrin-laminin interactions mediated the migration of ALL cells towards the cerebrospinal fluid in vitro. Mice with ALL xenografts were treated with either a PI3Kδ inhibitor, which decreased α6 integrin expression on ALL cells, or specific α6 integrin-neutralizing antibodies and showed significant reductions in ALL transit along bridging vessels, blast counts in the cerebrospinal fluid and CNS disease symptoms despite minimally decreased bone marrow disease burden. Our data suggest that α6 integrin expression, which is common in ALL, allows cells to use neural migratory pathways to invade the CNS.
急性淋巴细胞白血病(ALL)有明显向中枢神经系统(CNS)转移的倾向。与实体瘤的脑转移不同,ALL 的转移很少涉及实质,而是局限于软脑膜,这是癌性浸润的罕见部位。尽管 ALL 的所有亚型都有向 CNS 转移的情况,但尚未确定侵袭的统一机制。在这里,我们表明,循环中的 ALL 细胞无法穿透小鼠的血脑屏障;相反,它们沿着直接穿过椎骨或颅骨骨髓和蛛网膜下腔的血管迁移到 CNS。这些桥接血管的基底膜富含层粘连蛋白,层粘连蛋白已知协调 CNS 中神经元祖细胞的寻路。大多数 ALL 病例都表达层粘连蛋白受体α6 整合素。我们发现,α6 整合素-层粘连蛋白相互作用介导了 ALL 细胞在体外向脑脊液的迁移。用 PI3Kδ 抑制剂或特异性α6 整合素中和抗体治疗 ALL 异种移植小鼠,均显著减少了 ALL 细胞沿桥接血管的转移、脑脊液中白血病细胞的数量以及 CNS 疾病症状,尽管骨髓疾病负担的减少程度最小。我们的数据表明,ALL 中常见的α6 整合素表达允许细胞利用神经迁移途径侵入 CNS。