儿童种痘水疱病样淋巴组织增生性疾病临床病理分析

Clinical and pathological analysis on hydroa vacciniforme-like lymphoproliferative disorder in children

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。

Purpose To discuss the clinical, histopathological characteristics, diagnosis, differential diagnosis and prognosis of hydroa vacciniforme-like lymphoproliferative disorder in children. Methods 6 cases of hydroa vacciniforme-like lymphoproliferative disorder were analyzed by molecular, histopathologieal and immunohistochemical testing. Clinical and follow-up information was obtained. The published relevant liter- atures were reviewed. Results 4 cases were boys, 2 case were girls. All the patients presented with erythema and blisters with fever for 1 month to 4 years. I-Iistopathologic examination showed an mild or moderate atypical lymphocytic infiltrate with angiotro- pism and angiocentricity, and scattered or dense lymphoid infil- tration throughout the dermis and subcutaneous tissue. Blisters or necrosis could be seen in the epidermis. The atypical lymphocytes were positive for CD2, CD3, CDS, CD7, CD43, TIA-1, CD4 or CD8, and negative for CD20, Pax-5. Only one case showed positive stain for CD56. The average positive rate of Ki- 67 in tumor ceils was 42. 3 %. Tumor cells positive for EBV encoded RNA （EBER） were detected in cutaneous infiltrates in 5 cases. Gene rearrangement of TCR was detected in 2 cases. 5 patients were available for follow-up examination and 1 patient was dead. Conclusion Hydroa vacciniforme-like lymphoprolif- erative disorder is a rare disease mainly occuring in children. Chronic active EBV infection has been associated with this disease. It may be a spectrum in terms of its clinical course, and may be benign, borderline and malignant. Pathological diagnosis should be closely combined with clinical data.