Tanière P, Thivolet-Béjui F, Vitrey D, Isaac S, Loire R, Cordier J F, Berger F
Laboratoire d'Anatomie Pathologique de l'Hôpital E. Herriot, Lyon, France.
Eur Respir J. 1998 Jul;12(1):102-6. doi: 10.1183/09031936.98.12010102.
Four cases of lymphomatoid granulomatosis are reported, three of them involving the lung. Histological features included a true angiocentric and angiodestructive polymorphic cellular proliferation. This included histiocytes, plasma cells, many reactive T-cells and rare large, atypical cells which were of the B phenotype. Epstein-Barr virus was detected in the atypical cells by in situ hybridization in three cases, with expression of both latent membrane proteins (LMP)-1 and Epstein-Barr nuclear antigen-2 in two cases and expression of only LMP-1 in the third case. Expression of both of these proteins suggests a defect in the T-cell-mediated immunity and that Epstein-Barr virus is not only a silent passenger but may also be involved in the pathogenesis of the disease. This could have implications for therapy.
报告了4例淋巴瘤样肉芽肿病,其中3例累及肺部。组织学特征包括真正的血管中心性和血管破坏性多形性细胞增殖。这包括组织细胞、浆细胞、许多反应性T细胞以及罕见的大的非典型细胞,这些非典型细胞为B表型。通过原位杂交在3例病例的非典型细胞中检测到爱泼斯坦-巴尔病毒,2例病例中潜伏膜蛋白(LMP)-1和爱泼斯坦-巴尔核抗原-2均有表达,第3例病例中仅LMP-1有表达。这两种蛋白的表达提示T细胞介导的免疫存在缺陷,且爱泼斯坦-巴尔病毒不仅是无症状的过客,还可能参与了该疾病的发病机制。这可能对治疗有影响。