發(fā)布時間：2018-07-10 19:38

  本文選題：種痘水皰病 + 淋巴瘤��； 參考：《臨床與實驗病理學(xué)雜志》2017年09期

【摘要】：目的探討兒童種痘水皰病樣淋巴組織增生性疾病的臨床病理學(xué)特征、診斷、鑒別診斷及預(yù)后。方法收集6例兒童種痘水皰病樣淋巴組織增生性疾病,觀察其臨床表現(xiàn)、組織病理學(xué)、免疫表型及相關(guān)分子病理檢測,收集隨訪資料并復(fù)習(xí)相關(guān)文獻。結(jié)果男童4例,女童2例,病史1個月~4年。臨床均表現(xiàn)為反復(fù)發(fā)作的水痘樣皰疹并伴發(fā)熱。鏡下見表皮內(nèi)有水皰形成或伴壞死,真皮層至皮下脂肪層內(nèi)有數(shù)量多少不等的淋巴樣細胞浸潤,細胞可為輕至中度異型,多位于小血管及附屬器周圍。免疫組化示異型細胞可表達CD2、CD3、CD5、CD7、CD43、CD4、CD8、TIA-1,僅1例表達CD56,所有病例均不表達CD20、Pax-5。Ki-67增殖指數(shù)平均42.3%。5例EBER原位雜交檢測呈陽性,2例TCR克隆性重排陽性。5例獲得隨訪,1例患兒死亡。結(jié)論兒童種痘水皰病樣淋巴組織增生性疾病臨床較為少見,與慢性活動性EB病毒感染密切相關(guān),臨床過程可能為獨立的疾病譜系,其性質(zhì)可為良性、交界性及惡性。病理診斷需密切結(jié)合臨床表現(xiàn)。
[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.
【作者單位】： 廣州中醫(yī)藥大學(xué)第一附屬醫(yī)院病理科;廣州中醫(yī)藥大學(xué)第一附屬醫(yī)院皮膚科;廣州中醫(yī)藥大學(xué)第一附屬醫(yī)院病理科廣州510405;
【分類號】：R752

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