Tao J, Kahn L
Department of Pathology, Long Island Jewish Medical Center, The Long Island Campus for the Albert Einstein College of Medicine, New Hyde Park, NY 11040, USA.
Arch Pathol Lab Med. 2000 Oct;124(10):1520-4. doi: 10.5858/2000-124-1520-EBVAHG.
We report an unusual case of Epstein-Barr virus (EBV)-associated mucosal-associated lymphoid tissue (MALT) lymphoma involving the lungs, kidneys, and axillary lymph nodes in a child with congenital hypoadrenalism and panhypopituitarism. The patient presented with an aggressive clinical course and histologic evolution. Initial biopsies (1994) of the lung and kidney revealed histologic features of low-grade B-cell MALT lymphoma with lymphoepithelial lesions within the renal tubules and bronchial epithelium. Subsequent biopsies (1996, 1997, and 1999) revealed progressively greater cytologic atypia, polymorphism, and necrosis; an increased mitotic rate; and a preponderance of large cells, indicative of progression from a low-grade to a high-grade MALT lymphoma. Immunophenotyping of the lung and lymph node lesions revealed identical surface marker profiles: cells were CD19(+), CD20(+), immunoglobulin (Ig) G(+), kappa(+), lambda(-), CD5(-), CD10(-), CD23(-), and IgM(-), and also negative for T-cell markers. Genotypic analysis demonstrated the presence of immunoglobulin heavy chain rearrangement and monoclonality of EBV in the lung lesion by Southern blot hybridization and polymerase chain re()action (PCR). The clinicopathologic features suggest that these lesions might represent an immunosupression-related continuum of low-grade to high-grade MALT lymphomas. Infection with EBV may have contributed to this tumor's aggressive clinical and histologic evolution.
我们报告了一例罕见的爱泼斯坦-巴尔病毒（EBV）相关的黏膜相关淋巴组织（MALT）淋巴瘤病例，该病例发生在一名患有先天性肾上腺皮质功能减退症和全垂体功能减退症的儿童身上，累及肺部、肾脏和腋窝淋巴结。患者呈现出侵袭性的临床病程和组织学演变。最初（1994年）对肺和肾脏的活检显示为低度B细胞MALT淋巴瘤的组织学特征，肾小管和支气管上皮内有淋巴上皮病变。随后的活检（1996年、1997年和1999年）显示细胞异型性、多形性和坏死逐渐增加；有丝分裂率增加；大细胞占优势，表明从低度MALT淋巴瘤进展为高度MALT淋巴瘤。对肺和淋巴结病变的免疫表型分析显示表面标志物谱相同：细胞为CD19(+)、CD20(+)、免疫球蛋白（Ig）G(+)、κ(+)、λ(-)、CD5(-)、CD10(-)、CD23(-)、IgM(-)，并且对T细胞标志物也呈阴性。基因分析通过Southern印迹杂交和聚合酶链反应（PCR）证明肺病变中存在免疫球蛋白重链重排和EBV单克隆性。临床病理特征表明这些病变可能代表与免疫抑制相关的低度到高度MALT淋巴瘤的连续过程。EBV感染可能促成了该肿瘤侵袭性的临床和组织学演变。
