Division of Hematology/Oncology, Department of Medicine, Kameda Medical Center, Kamogawa-shi, Chiba, Japan.
Cancer. 2011 Oct 1;117(19):4512-21. doi: 10.1002/cncr.26090. Epub 2011 Mar 29.
Intravascular large B-cell lymphoma (IVL) is characterized by lymphoma cell proliferation in the lumina of small vessels in various organs. A high incidence of neurologic symptoms associated with the central nervous system has been reported, but peripheral nerve involvement (neurolymphomatosis [NL]) rarely has been described.
The medical records from patients who were diagnosed with IVL over the past 4 years were reviewed. A diagnosis of NL was made based on the combination of neurologic symptoms and their correspondence with imaging studies, such as magnetic resonance imaging (MRI), (18) F-fluoro-2-deoxy-D-glucose (FDG)-positron emission tomography/computed tomography (PET/CT), and/or the histologic confirmation of lymphoma cells within the peripheral nerves, nerve root/plexuses, or cranial nerves.
Four patients with NL were identified among 11 patients who had IVL. All cases of NL occurred as relapsed disease during or shortly after the completion of chemotherapy. Although MRI studies of the brains and whole spines revealed nerve infiltration by gadolinium enhancement in 2 patients, the technology was not sensitive enough to detect such infiltration in the remaining 2 patients. In contrast, FDG-PET/CT studies successfully revealed cranial or peripheral nerve lesions in all 4 patients and was useful for evaluating therapeutic response. Patients received treatment with high-dose methotrexate with or without other systemic chemotherapy, which achieved varied success. Further studies will be needed to determine the optimal treatment.
Considering the rarity of IVL and NL, the current observations suggested that IVL may have a predilection not only for the vessels but also for both the central and peripheral nervous systems.
血管内大 B 细胞淋巴瘤（IVL）的特征是在各种器官的小血管腔内发生淋巴瘤细胞增殖。据报道，该疾病与中枢神经系统相关的神经症状发生率较高，但很少有周围神经受累（神经淋巴组织病[NL]）的描述。
回顾了过去 4 年中诊断为 IVL 的患者的病历。根据神经症状及其与磁共振成像（MRI）、（18）F-氟代-2-脱氧-D-葡萄糖（FDG）-正电子发射断层扫描/计算机断层扫描（PET/CT）等影像学研究的对应关系，以及/或在外周神经、神经根/神经丛或颅神经中发现淋巴瘤细胞的组织学证实，诊断为 NL。
在 11 例 IVL 患者中发现了 4 例 NL。所有 NL 病例均发生在化疗完成期间或之后不久的复发疾病中。尽管 2 例患者的脑和全脊柱 MRI 研究显示钆增强神经浸润，但该技术不足以检测出另外 2 例患者的这种浸润。相比之下，FDG-PET/CT 研究成功地在所有 4 例患者中发现了颅神经或周围神经病变，并且对评估治疗反应有用。患者接受了大剂量甲氨蝶呤联合或不联合其他全身化疗的治疗，取得了不同程度的成功。需要进一步的研究来确定最佳治疗方法。
考虑到 IVL 和 NL 的罕见性，目前的观察结果表明，IVL 可能不仅倾向于血管，还倾向于中枢和周围神经系统。
