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血管免疫母细胞淋巴腺病(Angioimunoblastic Lymphadenopathy,简称AIL)是一种免疫功能异常的淋巴组织增生性疾病。其临床特点为全身浅表淋巴结肿大、发热、皮疹及肝脾肿大,部分病例可有多株高球蛋白血症或溶血性贫血等表现。淋巴结病理改变具有大量小血管增生,免疫母细胞出现及间质无定形嗜酸性物质沉着的“三联征”。本病临床经过急凶且常有恶变,预后较差。近年来,亦已引起国内有关临床学科的重视,并有陆续报道。我们最近收治一例,且有较显著皮肤、毛发病变,现予报告。
Angioimmunoblastic Lymphadenopathy (AIL) is a lymphoproliferative disease with abnormal immune function. The clinical features of superficial lymph nodes of the body, fever, rash and hepatosplenomegaly, in some cases may have multiple strains of hypergammaglobulinemia or hemolytic anemia and other performance. Pathological changes of lymph nodes with a large number of small blood vessel hyperplasia, immune cells and interstitial amorphous eosinophilic “triple sign.” The clinical emergency after the fierce and often have malignant, the prognosis is poor. In recent years, it has also drawn the attention of the relevant clinical disciplines in the country and reports successively. We recently admitted a case, and there are more significant skin, hair lesions, is reported.