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血管免疫母细胞性淋巴结病:光镜与电镜研究

Angioimmunoblastic lymphadenopathy: light and electron microscopic studies.

作者信息

Takahashi H, Wakasa H

出版信息

Tohoku J Exp Med. 1982 Mar;136(3):267-84. doi: 10.1620/tjem.136.267.

Abstract

The present study evaluates the significance of clinical and histologic features in 16 cases of angioimmunoblastic lymphadenopathy (AIL). The disease observed in the elderly is characterized by an acute onset of constitutional symptoms, generalized lymphadenopathy, hepatosplenomegaly, and immunologic abnormalities. The clinical course is rather short, and autopsied cases show severe infections complications. In the present study, two histologic types were identified: 1) Angio-cellular (A-C) type: the lymph nodes contained lymphocytes, immunoblasts, histiocytes, eosinophils and/or plasma cells. 2) Immunoblastic and plasma cell (I & P) type or lymphocyte depleted (LD) type; the lymph node contained mainly immunoblasts and plasmocytoid cells. Serum protein was examined in 12 cases. Dysproteinemia was noted in 5 of 6 cases of A-C type and in 5 of 6 cases of I & P type. There is a definite relationship between histologic types and survival periods. The A-C type is an expression of slight depletion of lymphocytes and the I & P or LD type represents marked depletion of lymphocytes. AIL has malignant potentiality, though it resembles graft-vs-host reaction in histology, the wide-spread involvement of tissues and clinical features. An immunologic investigation on 3 cases suggests that the disease is based on B-cell disorder.

摘要

本研究评估了16例血管免疫母细胞性淋巴结病(AIL)的临床和组织学特征的意义。在老年人中观察到的这种疾病的特点是全身症状急性发作、全身淋巴结肿大、肝脾肿大和免疫异常。临床病程相当短,尸检病例显示有严重的感染并发症。在本研究中,确定了两种组织学类型:1)血管细胞(A-C)型:淋巴结含有淋巴细胞、免疫母细胞、组织细胞、嗜酸性粒细胞和/或浆细胞。2)免疫母细胞和浆细胞(I&P)型或淋巴细胞耗竭(LD)型;淋巴结主要含有免疫母细胞和浆细胞样细胞。对12例患者进行了血清蛋白检查。在A-C型的6例中有5例以及I&P型的6例中有5例出现了蛋白异常血症。组织学类型与生存期之间存在明确的关系。A-C型是淋巴细胞轻度耗竭的表现,而I&P或LD型则代表淋巴细胞明显耗竭。AIL具有恶性潜能,尽管它在组织学、广泛的组织受累和临床特征方面类似于移植物抗宿主反应。对3例患者的免疫研究表明,该疾病基于B细胞紊乱。

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