成年人系统性EB病毒阳性T／NK细胞淋巴组织增殖性疾病的临床病理研究  

Clinicopathologic features of systemic EBV-positive T/NK-cell lymphoproliferative disease in adults

作　　者：郑晓丹[1] 周小鸽[1] 金妍[1] 谢建兰[1] 魏雪静[1] 陈书媛[1] 梅雪[1] 宫丽平[2] 吕蓓蓓[2] 

机构地区：[1]首都医科大学附属北京友谊医院病理科,100050 [2]首都医科大学病理学教研室

出　　处：《中华病理学杂志》2011年第4期227-234,共8页Chinese Journal of Pathology

摘　　要：目的探讨成年人系统性EB病毒阳性T／NK细胞淋巴组织增殖性疾病（ASEBV＋T／NK—LPD）的临床病理特征、免疫表型和EBV感染特征，以提高对其的认识及诊断水平。方法经过HE染色观察并采用免疫组织化学、原位杂交及基因重排技术，结合临床资料，对20例ASEBV＋T／NK—LPD患者病理资料进行回顾性研究，并进行随访。结果（1）男15例，女5例，中位年龄34岁；起病至确诊时间平均8．7个月；主要临床表现有发热（18／20）、肝脾大（18／20）、淋巴结肿大（17／20）；17例随访中11例死亡，平均生存时间2．9个月。（2）组织病理学特点：淋巴结T区明显增宽至弥漫成片，淋巴滤泡不同程度地减少或消失。病变中细胞种类多样至相对单一，以中小淋巴细胞增多为主，大细胞散在其中。淋巴细胞多数异型性不明显，少数轻或中度异型。6例被膜增厚，9例可见局灶或片状坏死及核碎；3例可见噬血细胞现象；另外，肝、脾、肠黏膜、扁桃体和骨髓主要表现为轻至中度异型淋巴细胞浸润。（3）免疫表型特点：20例都表现出T细胞明显多于B细胞，很多细胞表达颗粒酶B或T细胞胞质内抗原（TIA）-1，仅1例CD56阳性。14例CD4／CD8检测病例中8例以CD8阳性细胞为主，5例以CD4阳性细胞为主，i例CD8和CD4阳性数量相当。（4）全部病例EBER均阳性。阳性细胞最多的区域数量在30～300个／HPF，绝大多数病例中EBER阳性细胞包括大、中、小淋巴细胞，主要分布在扩大的T区。（5）TCR基因重排显示该病细胞群体中既有多克隆（4／7）也有单克隆（3／7）。结论ASEBV＋T／NK—LPD呈亚急性或慢性经过，主要表现为反复发热伴淋巴结及肝脾大；淋巴结以T区扩大和轻中度异型淋巴细胞浸润为主；EBV感染的细胞毒性T细胞构成病变主体。它是一种危及生命的疾病，患者往往死于器官衰竭和出血。Objective To study the clinicopathologic features, immunophenotype, clonality and Epstein-Barr virus （EBV） status of systemic EBV-positive T/NK-cell lymphoproliferative disease in adults （ASEBV ＋T/NK-LPD）. Methods Twenty cases of ASEBV ＋T/NK-LPD were analyzed retrospectively with histopathologic review, immunohistochemistry and in-situ hybridization for EBV-encoded RNA （EBER）. The follow-up data were collected. Results There were altogether 15 males and 5 females. The median age of the patients was 34 years. The average duration from onset of symptoms to diagnosis was 8.7 months. Fever （18/20）, hepatosplenomegaly （18/20） and lymphadenopathy （17/20） were the main clinical manifestations. Eleven of the 17 patients died during follow-up, with a mean survival of 2. 9 months. Histologically, there was obvious expansion of T zone of the involved lymph nodes, associated with diminished lymphoid follicles. The interfollicular areas were widened and infiltrated by small to median-sized lymphoid cells which showed only mild atypia. Scattered large lymphoid cells were not uncommon. The nodal capsule was thickened in 6 cases. Focal necrosis was seen in 9 cases. Sinus histiocytic proliferation with erythrophagocytosis was observed in 3 cases. In addition, there were mild atypical lymphoid cells infiltrate into the liver, spleen, intestinal mucosa and bone marrow. Immunohistochemical study and in-situ hybridization showed that the EBER-positive cells were of T-cell lineage, with CD3 expression. They were also positive for cytotoxic molecules （granzyme B or TIA-1 ） . Only 1 case was CD56 positive. A predominance of CDS-positive cells was demonstrated in 8 of the 14 cases studied, while CD4-positive cells predominated in the remaining 5 cases. One case showed similar proportion of CD8 and CD4-positive cells. The number of EBER-positive cells ranged from 30 to more than 300 per high-power fields. These EBER-positive cells were of small to large size and located mainly in the expanded T zone and occ

关 键 词：疱疹病毒4型 人 淋巴组织增殖性疾病 T淋巴细胞 成年人 免疫表型分型 原位杂交 

分 类 号：R5[医药卫生—内科学]