Heesen C, Bergmann M, Figge C, Löschke S, Feldmann M
Neurologische Klinik, Universität Hamburg.
Fortschr Neurol Psychiatr. 1996 Jun;64(6):234-41. doi: 10.1055/s-2007-996391.
We report on a typical case of intravascular lymphomatosis, a rarely diagnosed, generalised intravascular lymphoma usually of the B-cell type. In most cases there is a lack of clear haematological findings but in more than 50% intravascular lymphomatosis presents with symptoms of the central nervous system. Every rapidly progressive neurological deficit, especially the association of a subacute dementia with a spinal syndrome may suggest IVL-NS. However, careful examination may detect minor features for a systemic process in 25-80% i.e. B-symptoms elevation of ESR and LDH. Neurological imaging demonstrates multifocal lesions in the CNS with affinity to the deep white matter consistent with a microvascular or demyelinating disease. Angiographically IVL-NS may mimic cerebral vasculitis. CSF findings are nonspecific. Because diagnosis can only be made histologically, a cerebral biopsy should be undertaken in suspected cases. Usually the course of the disease is fatal. Therapy involving steroids, combination polychemotherapy or radiation met with only minor success.
我们报告了一例典型的血管内淋巴瘤病例，这是一种罕见的、通常为B细胞型的全身性血管内淋巴瘤。大多数情况下，缺乏明确的血液学表现，但超过50%的血管内淋巴瘤患者会出现中枢神经系统症状。每一例快速进展的神经功能缺损，尤其是亚急性痴呆与脊髓综合征的关联，都可能提示中枢神经系统血管内淋巴瘤（IVL-NS）。然而，仔细检查可能会在25%至80%的患者中发现系统性病变的细微特征，即B症状、血沉（ESR）和乳酸脱氢酶（LDH）升高。神经影像学显示中枢神经系统有多发性病变，与深部白质相关，符合微血管或脱髓鞘疾病。血管造影显示IVL-NS可能类似脑血管炎。脑脊液检查结果无特异性。由于只能通过组织学诊断，疑似病例应进行脑活检。通常，该病的病程是致命的。使用类固醇、联合多药化疗或放疗的治疗仅取得了微小的成功。
