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目的探讨小儿骨朗格汉斯细胞组织细胞增生症(LCH)的临床特点、病理形态学、免疫表型特征、鉴别诊断及预后。方法回顾性分析108例小儿骨LCH的临床资料,其中40例行免疫组织化学染色。按2000年WHO分类标准重新分类,并随访。结果108例小儿骨LCH中累及单块骨73.14%(79/108例),累及多块骨(伴或不伴皮肤受累)25%(27/108例),累及多个脏器(骨、肝、脾及其他)1.9%(2/108例)。累及单块骨及多块骨5a生存率均为100%,累及多个脏器2例,随访25个月2例均死亡。免疫组织化学染色显示,40例小儿骨LCH中CD1a阳性100%(40/40例),Vim阳性90%(36/40例),S100阳性90%(36/40例),CD68阳性67.5%(27/40例),Lys阳性40%(16/40例),MAC387阳性30%(12/40例),CK、EMA均阴性。结论小儿骨LCH是一种病因不明的肿瘤性增生性疾病,在病理学上具有特殊的形态学表现及免疫组织化学表型。疾病的预后与骨病损范围和病理分型有关,大部分病例预后好。
Objective To investigate the clinical features, histopathology, immunophenotypic characteristics, differential diagnosis and prognosis of children with Bengal Gehuns cell histiocytosis (LCH). Methods A retrospective analysis of 108 cases of pediatric bone LCH clinical data, of which 40 cases were immunohistochemical staining. According to 2000 WHO classification criteria reclassified, and follow-up. Results Of 108 pediatric patients with LCH, 73.14% (79/108) of the mononuclear bone masses were involved, involving 25% (27/108 cases) of multiple bones (with or without skin involvement) involving multiple organs (bone and liver , Spleen and others) 1.9% (2/108 cases). Survival rates of 100% involving single bones and multiple bones 5a, involving multiple organs in 2 cases, were followed up for 25 months in 2 cases were dead. Immunohistochemical staining showed that the positive rate of CD1a was 100% (40/40), Vim was 90% (36/40), S100 was 90% (36/40) and CD68 was 67.5% 27/40 cases), Lys positive 40% (16/40 cases), MAC387 positive 30% (12/40 cases), CK, EMA were negative. Conclusion Pediatric bone LCH is a neoplasm of unknown etiology and has special morphological features and immunohistochemical phenotype in pathology. The prognosis of the disease and the extent of bone lesions and pathological classification, the prognosis of most cases is good.