Bunn P A, Schein P S, Banks P M, DeVita V T
Blood. 1976 Jan;47(1):3-10.
Fifteen of 52 patients (29%) with diffuse histiocytic and undifferentiated pleomorphic lymphoma developed central nervous system (CNS) complications, primarily leptomeningeal lymphoma. Lumbar puncture with cerebrospinal fluid cytology was the most useful test for diagnosis, and for following the response to therapy. Leptomeningitis developed during all stages of the patients' clinical course: at time of diagnosis, during progression of systemic disease, and most importantly as the initial site of relapse within 7 mo of attaining a complete clinical remission. Patients with bone marrow involvement are at high risk for the development of leptomeningeal lymphoma. Pathologic findings suggest that entry into the leptomeninges involves extension from the medullary bone marrow cavity along perforating vessels through dura into the arachnoid space. The leptomeningeal lymphoma has been successfully controlled in all patients receiving intensive central nervous system therapy consisting of a combination of intrathecal drug administration and radiotherapy. The high frequency of this syndrome and the success in its control suggest that a controlled trial of prophylactic CNS therapy be instituted in patients with these histologic types of non-Hodgkin's lymphomas.
52例弥漫性组织细胞性和未分化多形性淋巴瘤患者中有15例（29%）发生中枢神经系统（CNS）并发症，主要是软脑膜淋巴瘤。腰椎穿刺及脑脊液细胞学检查是诊断及监测治疗反应最有用的检查。软脑膜炎在患者临床病程的各个阶段均可发生：诊断时、全身疾病进展期间，最重要的是在完全临床缓解后7个月内作为复发的初始部位。骨髓受累的患者发生软脑膜淋巴瘤的风险很高。病理结果提示，进入软脑膜涉及从骨髓腔沿穿通血管穿过硬脑膜延伸至蛛网膜下腔。所有接受由鞘内给药和放疗联合组成的强化中枢神经系统治疗的患者，其软脑膜淋巴瘤均得到成功控制。该综合征的高发生率及其控制的成功表明，应对这些组织学类型的非霍奇金淋巴瘤患者进行预防性中枢神经系统治疗的对照试验。
