Marelle L, Raphaël M, Henin D, Vazeux R, Schuller E, Piette J C, Poisson M, Gentilini M, Hauw J J
Laboratoire de Neuropathologie R. Escourolle, Centre d'Ecologie cellulaire, Département d'Hématologie, Hôpital de La Salpêtrière, Paris.
Rev Neurol (Paris). 1994;150(2):123-32.
We report the natural history of 17 brain lymphomas (11 primary, 6 disseminated) from a post-mortem series of 130 patients with AIDS. Primary lymphomas appeared lately in the course of AIDS. They were often associated with a severe T-cell immunodepression and with more frequent opportunistic disorders than disseminated lymphomas. Associated Kaposi's sarcomas were surprisingly frequent. All patients presented with neurological manifestations. Heterogeneous features were seen at CT examination. The CSF was abnormal in 12/13 cases, with an increase of protein contents and secretion of immunoglobulins; it contained activated lymphocytes in 5/6 cases of disseminated lymphomas, and malignant cells in only one case. Cellular density never exceeded 8/mm3 for primary lymphomas, and the lymphocytes were considered normal. The pre-mortem diagnosis of cerebral lymphomas was made in five patients, with a time lapse of 1 to 7 months between the first neurological symptoms and death, and of 5 to 30 days between the diagnosis and death. Cerebral biopsy was diagnostic in 4 cases of primary cerebral lymphomas. In only 1/6 patients with disseminated lymphomas, the diagnosis had been made when the patient was still alive, based on CSF and bone marrow lymphomatous infiltrations. The diagnosis of cerebral lymphoma (7 primary, 5 disseminated) was post-mortem in 12 cases. It was made only at microscopic examination in 2/12 cases of primary lymphomas. The histopathological study frequently showed a multicentric involvement, and always an immunoblastic cell type with plasmablastic differentiation and frequent medium size cells. Marked gliosis and significant necrosis were often observed. Neuropathological lesions associated with HIV-1 infection (toxoplasmosis, CMV and HIV-1 encephalitis) were seen in 8 cases with primary lymphomas.
我们报告了130例艾滋病患者尸检系列中的17例脑淋巴瘤（11例原发性，6例播散性）的自然病史。原发性淋巴瘤在艾滋病病程中出现较晚。它们常与严重的T细胞免疫抑制相关，且与播散性淋巴瘤相比，机会性疾病更为常见。相关的卡波西肉瘤出人意料地频繁出现。所有患者均有神经学表现。CT检查可见异质性特征。13例中有12例脑脊液异常，蛋白质含量增加，免疫球蛋白分泌增多；6例播散性淋巴瘤中有5例脑脊液中含有活化淋巴细胞，仅1例含有恶性细胞。原发性淋巴瘤的细胞密度从未超过8/mm³，淋巴细胞被认为是正常的。5例患者在生前做出了脑淋巴瘤的诊断，从首次出现神经症状到死亡的时间间隔为1至7个月，从诊断到死亡的时间间隔为5至30天。4例原发性脑淋巴瘤经脑活检确诊。6例播散性淋巴瘤患者中只有1例在患者仍存活时，根据脑脊液和骨髓淋巴瘤浸润做出了诊断。12例患者的脑淋巴瘤（7例原发性，5例播散性）诊断是在尸检时做出的。12例原发性淋巴瘤中有2例仅在显微镜检查时做出诊断。组织病理学研究经常显示多中心受累，且总是免疫母细胞型，伴有浆母细胞分化，中等大小细胞常见。经常观察到明显的胶质增生和显著的坏死。8例原发性淋巴瘤患者可见与HIV-1感染相关的神经病理学病变（弓形虫病、巨细胞病毒和HIV-1脑炎）。
