Dubas J M, Widgren S, Zwahlen A
Schweiz Med Wochenschr. 1978 Aug 26;108(34):1295-302.
Eight cases of angio-immunoblastic lymphadenopathy were recently diagnosed at the University Hospital of Geneva. The disease was characterized by generalized adenopathy, hepatosplenomegaly, fever, and sometimes skin rash. Six patients had polyclonal dysproteinemia and two displayed autoimmune disorders, e.g. Coombs' positive hemolytic anemia. Lymph node biopsy was a decisive pointer for the diagnosis. Four patients died, two of them from immunoblastic lymphoma. Four other patients are in remission, the longest follow-up being 91 months. Analysis of 213 cases from the literature shows the following features:--Males and females are equally affected, with a predominance after the age of 50.--The onset is characterized by constitutional symptoms (75%) and fever (65%), sometimes following exposure to drugs.--The signs are generalized adenopathy (91%), splenomegaly (66%), hepatomeagly (65%), and skin rash (46).--Polyclonal hypergammaglobulinemia (76%) is found, together with anemia (78%) which is often autoimmune in origin (58%).--The course is often unfavourable and the mortality is over 55%, largely due to opportunistic infections and/or development of immunoblastic lymphoma.
日内瓦大学医院最近诊断出8例血管免疫母细胞性淋巴结病。该病的特征为全身性淋巴结肿大、肝脾肿大、发热，有时伴有皮疹。6例患者有多克隆性蛋白异常血症，2例表现为自身免疫性疾病，如抗人球蛋白试验阳性的溶血性贫血。淋巴结活检是诊断的决定性依据。4例患者死亡，其中2例死于免疫母细胞性淋巴瘤。其他4例患者病情缓解，最长随访时间为91个月。对文献中213例病例的分析显示出以下特征：——男性和女性受影响程度相同，50岁以后更为多见。——起病时以全身症状（75%）和发热（65%）为特征，有时在接触药物后出现。——体征为全身性淋巴结肿大（91%）、脾肿大（66%）、肝肿大（65%）和皮疹（46%）。——发现多克隆性高球蛋白血症（76%），以及贫血（78%），贫血常源于自身免疫（58%）。——病程通常不利，死亡率超过55%，主要是由于机会性感染和/或免疫母细胞性淋巴瘤的发展。
