种痘水疱病样淋巴组织增生性疾病11例临床病理分析  

Clinicopathological analysis of 11 cases of hydroa vacciniforme-like lymphoproliferative disease

作　　者：白玄业 李文才[1] 王冠男[1] 赵武干[1] 陈旭[1] BAI Xuan-ye;LI Wen-cai;WANG Guan-nan;ZHAO Wu-gan;CHEN Xu(Department of Pathology,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,Chin)

机构地区：[1]郑州大学第一附属医院病理科,郑州450052

出　　处：《临床与实验病理学杂志》2018年第7期764-768,共5页Chinese Journal of Clinical and Experimental Pathology

基　　金：河南省重大科技专项子课题(161100311400);河南省医学科技攻关计划普通项目(201303016)

摘　　要：目的探讨种痘水疱病样淋巴组织增生性疾病(hydroa vacciniforme-like lymphoproliferative disease,HV-LPD)的临床病理学特点、诊断及鉴别诊断。方法收集11例HV-LPD的临床资料并进行形态学观察,应用免疫组化、EB病毒(Epstein-Barr virus,EBV)原位杂交检测和T细胞受体基因重排检测,并复习相关文献。结果 11例患者发病年龄2~50岁,临床表现为头面部和(或)躯干部位的肿胀、水疱、斑丘疹、结痂、瘢痕数月至数年,伴或不伴发热、肝脾及淋巴结肿大等症状。病理形态学示淋巴样细胞浸润真皮或至皮下组织,细胞小至中等大,轻至中度异型性,围绕皮肤附件浸润和嗜血管生长。11例病变细胞CD3、CD4均阳性,TIA-1(9/11)、CD8(8/11)、Granzyme B(6/11)、CD56(1/11)阳性,EBER均阳性。7例标本TCR克隆性重排检测阳性。11例患者临床治疗方案不同,包括抗病毒、抗感染、激素、免疫抑制剂、化疗,5例患者病情稳定,2例患者病情持续,1例死亡,3例失访。结论 HV-LPD是发生于儿童的EBV相关性T细胞淋巴组织增生性疾病,临床少见,亦可见于成人,其表现多样且独特,临床病史尤其以皮损特点是重要的诊断线索,结合病理形态、免疫表型、EBV原位杂交、T细胞受体基因重排可确诊。Purpose To analyze the clinical and pathologi- cal features of hydroavacciniforme-like lymphoproliferative disease （HV-LDP）. Methods 11 cases of HV-LDP including clinical manifestation, pathological characteristics, immunohistochemical staining, detection of EBV and T-cell receptor （TCR） gene rearrangement were studied. Results The patients aged from 2 years to 50 years, who presented with swell- ing, blister, maculopapule, escharosis, cicatrices on the head or trunk formonthes and years. Fever, lympbadenopathy, and hepatosplenomegaly could be observed. Histopathological examination showed a small or medium sized variable atypical lymphocytes infiltration in the dermis or subcutaneous, with periadnexal and perivascular involving. Immunohistochmcial staining showed the atypical lymphocytes of 11 cases were positive for CD3 and CD4, 9 cases for TIA-1, 8 cases for CD8, 6 cases for Granzyme B, only 1 case for CD56. All cases were EBER-positive and 7 case showed monoclonal T-cell receptor rearrangement. All cases varied in clinical therapy, which contain antiviral therapy, anti-infection, hormonotheraphy, immunosuppressive and chemotherapy. 5 patients were in stable conditions, 2 patients were persistent, 1 died, and 3 were lost to follow-up. Conclusion HV-LDP is a rare EBV-associated T cell lymphoproliferative disease that typically affects children and can also seen in aduluts. Its manifestations are diverse and unique. The clinical history, especially with skin lesion, is an important diagnostic clue. It can be confirmed by the combination of pathologic morphology, immunophenotype, EBV in situ hybridization, as well as TCR gene rearrangement.

关 键 词：T细胞淋巴组织增生性疾病 种痘水疱病样 EB病毒 

分 类 号：R733.4[医药卫生—肿瘤]