Negri Gualdi C
Minerva Med. 1978 Sep 19;69(43):2943-9.
The clinico-histologico-biological picture of a recently identified haematological condition distinguished by marked superficial generalized adenomegaly, a typical lymph node biopsy report (lively immunoblastic and angioblastic proliferation, deposits of PAS-positive eosinophil homogenous material) and hypergammaglobulinaemia (polyclonal gammopathy) and defined as angio-immunoblastic lymphadenopathy with dysproteinaemia, is described. On the basis of numerous reported cases and the clinical case in question, the pathogenetic interpretation whereby the condition is considered as a reaction in similar fashion to what is already known for the so-called GvHr (transplant to host reaction), independently of the as yet unknown and perhaps many-sided aetiological factor, is upheld. The condition is substantially benign, although the possibility that it will become malignant cannot be excluded. It is highly sensitive to radiant, cytostatic and cortiocosteroid therapy although there are profound prognostic reservations about its great susceptibility to infections, particularly at respiratory level.
描述了一种最近发现的血液学病症的临床-组织学-生物学特征,其特点为明显的全身性浅表淋巴结肿大、典型的淋巴结活检报告(活跃的免疫母细胞和血管母细胞增殖、PAS阳性嗜酸性均匀物质沉积)以及高球蛋白血症(多克隆丙种球蛋白病),该病症被定义为伴有蛋白异常血症的血管免疫母细胞性淋巴结病。基于大量已报道病例及所讨论的临床病例,支持如下发病机制解释:该病症被视为与所谓的移植物抗宿主反应(GvHr)已知情况类似的一种反应,而与尚未明确且可能是多方面的病因因素无关。该病症本质上是良性的,尽管不能排除其恶变的可能性。它对放疗、细胞抑制疗法和皮质类固醇疗法高度敏感,不过鉴于其对感染,尤其是呼吸道感染的高度易感性,对其预后存在很大保留意见。