Cramer S C, Glaspy J A, Efird J T, Louis D N
Neurology Service, Massachusetts General Hospital, Boston 02114, USA.
Neurology. 1996 Jan;46(1):19-25. doi: 10.1212/wnl.46.1.19.
Chronic lymphocytic leukemia is the most common human leukemia but infrequently causes neurologic symptoms. We have reviewed all previously reported cases of chronic lymphocytic leukemia in the CNS along with three new cases; one patient was diagnosed antemortem and treated with immediate improvement and 4-year survival. In addition, we reviewed all autopsy cases since 1972 and available lumbar puncture data on patients with chronic lymphocytic leukemia admitted to the Massachusetts General Hospital. Invasion of the CNS by chronic lymphocytic leukemia often leads to confusional state, meningitis with cranial nerve abnormalities, optic neuropathy, or cerebellar dysfunction. Lumbar puncture shows a lymphocytosis consisting of monoclonal B cells, but CSF cytology studies are of limited value in establishing the diagnosis. Long-term survival may be related to the stage of chronic lymphocytic leukemia at the time of CNS disease and may be associated with intrathecal chemotherapy. A mild, asymptomatic infiltration of the brain, frequently noted in late-stage chronic lymphocytic leukemia in autopsy series, may explain the CSF lymphocytosis in some patients with late-stage chronic lymphocytic leukemia.
慢性淋巴细胞白血病是最常见的人类白血病，但很少引起神经系统症状。我们回顾了所有先前报道的中枢神经系统慢性淋巴细胞白血病病例以及3例新病例；1例患者生前确诊并接受治疗，症状立即改善，存活了4年。此外，我们回顾了自1972年以来的所有尸检病例以及麻省总医院收治的慢性淋巴细胞白血病患者的腰椎穿刺数据。慢性淋巴细胞白血病侵犯中枢神经系统常导致意识模糊状态、伴有脑神经异常的脑膜炎、视神经病变或小脑功能障碍。腰椎穿刺显示淋巴细胞增多，由单克隆B细胞组成，但脑脊液细胞学检查在确立诊断方面价值有限。长期存活可能与中枢神经系统疾病发生时慢性淋巴细胞白血病的分期有关，可能与鞘内化疗有关。在尸检系列中，晚期慢性淋巴细胞白血病患者常可见轻度、无症状的脑浸润，这可能解释了一些晚期慢性淋巴细胞白血病患者的脑脊液淋巴细胞增多现象。
