Ankita Grover, Shashi Dhawan
Department of histopathology, Sir Gangaram Hospital, Ist floor, SSRB Block. Rajender Nagar, New Delhi, India.
Indian J Surg Oncol. 2016 Dec;7(4):484-487. doi: 10.1007/s13193-016-0525-1. Epub 2016 Apr 29.
Lymphomatoid granulomatosis is a rare, Epstein Barr Virus (EBV)-associated systemic angiodestructive disorder that may progress to a diffuse large B cell lymphoma. Pulmonary involvement occurs in over 90 % cases followed by kidney, skin and brain. WHO classifies lymphomatoid granulomatosis under the generic heading of B cell proliferations of uncertain malignant potential. Radiologically, pulmonary lymphomatoid granulomatosis (PLG) presents with non specific findings making histopathology the gold standard for diagnosis. The histological diagnosis of PLG includes a triad of polymorphic lymphoid infiltrates, transmural infiltration of arteries and veins by lymphoid cells ("angiitis"), and focal areas of necrosis within the lymphoid infiltrates. PLG should be distinguished from granulomatosis with polyangitis, as well as other forms of malignant lymphoma, like extranodal NK/T cell lymphoma, secondary diffuse large B-cell lymphoma and primary Non Hodgkin lymphomas of lung.
淋巴瘤样肉芽肿病是一种罕见的、与爱泼斯坦-巴尔病毒(EBV)相关的系统性血管破坏性疾病,可进展为弥漫性大B细胞淋巴瘤。超过90%的病例会出现肺部受累,其次是肾脏、皮肤和脑部。世界卫生组织将淋巴瘤样肉芽肿病归类于恶性潜能不确定的B细胞增殖这一通用类别下。在放射学上,肺部淋巴瘤样肉芽肿病(PLG)表现为非特异性表现,这使得组织病理学成为诊断的金标准。PLG的组织学诊断包括多形性淋巴样浸润、淋巴样细胞对动脉和静脉的透壁浸润(“血管炎”)以及淋巴样浸润内的局灶性坏死三联征。PLG应与肉芽肿性多血管炎以及其他形式的恶性淋巴瘤相鉴别,如结外NK/T细胞淋巴瘤、继发性弥漫性大B细胞淋巴瘤和原发性肺非霍奇金淋巴瘤。