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1974年及1975年国外先后较系统地报告了数十例易与何杰金氏病等恶性淋巴瘤混淆的一种“临床病理综合征”,即血管——免疫母细胞性淋巴结病伴异常蛋白血症(Angio-immunoblastic Lymphadenopathy with Dys-proteinaemia AILD)或称血管——免疫母细胞性淋巴结病(AIL)。以后陆续有这方面的研究报导。此病临床象恶性过程,有发病凶急、全身浅表淋巴结肿大、发热、皮疹、脾、肝肿大、贫血、大部为多克隆性的球蛋白血症等表现;淋巴结病理组织象则有免疫母细胞增生、分枝状小血管增生及间质无定形嗜酸性物质沉着的“三联征”特点。本病预后不良。病因未明确。现重点综述近年来对此病淋巴结病理学及疾病本质的研究。
In 1974 and 1975, foreign countries have systematically reported dozens of cases of Hodgkin’s disease and other malignant lymphomas easily confused with a “clinicopathological syndrome”, that is, vascular-immune cell lymphadenopathy with abnormal protein Angio-immunoblastic Lymphadenopathy with Dys-proteinaemia AILD or angio-immunoblastic lymphadenopathy (AIL). After one after another in this area research reports. Clinical manifestations of the disease as a vicious process, there is a hurry, the whole body superficial lymph nodes, fever, rash, spleen, hepatomegaly, anemia, mostly polyclonal globulinmia and other manifestations; like lymph node histology There are immune cells proliferation, proliferation of small branches of blood vessels and interstitial amorphous eosinophilic “triple sign” characteristics. Poor prognosis of the disease. Etiology is not clear. Now focuses on the pathogenesis of this disease in recent years, pathology and disease nature of the study.