儿童种痘水疱病样淋巴组织增生性疾病临床病理分析

发布时间：2018-07-10 19:38

本文选题：种痘水疱病 + 淋巴瘤　；参考：《临床与实验病理学杂志》2017年09期

【摘要】：目的探讨儿童种痘水疱病样淋巴组织增生性疾病的临床病理学特征、诊断、鉴别诊断及预后。方法收集6例儿童种痘水疱病样淋巴组织增生性疾病,观察其临床表现、组织病理学、免疫表型及相关分子病理检测,收集随访资料并复习相关文献。结果男童4例,女童2例,病史1个月~4年。临床均表现为反复发作的水痘样疱疹并伴发热。镜下见表皮内有水疱形成或伴坏死,真皮层至皮下脂肪层内有数量多少不等的淋巴样细胞浸润,细胞可为轻至中度异型,多位于小血管及附属器周围。免疫组化示异型细胞可表达CD2、CD3、CD5、CD7、CD43、CD4、CD8、TIA-1,仅1例表达CD56,所有病例均不表达CD20、Pax-5。Ki-67增殖指数平均42.3%。5例EBER原位杂交检测呈阳性,2例TCR克隆性重排阳性。5例获得随访,1例患儿死亡。结论儿童种痘水疱病样淋巴组织增生性疾病临床较为少见,与慢性活动性EB病毒感染密切相关,临床过程可能为独立的疾病谱系,其性质可为良性、交界性及恶性。病理诊断需密切结合临床表现。
[Abstract]:Objective to investigate the clinicopathological features, diagnosis, differential diagnosis and prognosis of lymphoproliferative diseases of vaccinia varicella in children. Methods the clinical manifestations, histopathology, immunophenotype and molecular pathology of 6 children with vaccinia vesicular lymphoproliferative diseases were observed. The follow-up data were collected and the related literatures were reviewed. Results there were 4 boys and 2 girls with a history of 1 month to 4 years. The clinical manifestations were recurrent chickenpox herpes with fever. Microscopically there were blister formation or necrosis in the epidermis and infiltration of lymphoid cells from the dermis to the subcutaneous adipose layer. The cells could be mild to moderate dysplasia mostly located around the small blood vessels and appendages. Immunohistochemical staining showed that CD2, CD3, CD5, CD7, CD47, CD4, CD8, CD8, TIA-1 and CD56 were expressed in all cases, and no CD20 Pax-5.Ki-67 proliferative index was detected in all cases. The EBER in situ hybridization showed that 2 cases were positive for clonal rearrangement of TCR. 1 case was followed up. Conclusion Vaccine blister-like lymphoproliferative diseases in children are relatively rare and are closely related to chronic active Epstein-Barr virus (EBV) infection. The clinical process may be an independent disease lineage, and its nature may be benign, borderline and malignant. Pathological diagnosis should be closely combined with clinical manifestations.
【作者单位】：广州中医药大学第一附属医院病理科;广州中医药大学第一附属医院皮肤科;广州中医药大学第一附属医院病理科广州510405;
【分类号】：R752

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