骨朗格汉斯细胞组织细胞增生症108例临床病理分析

Clinicopathologic Analysis of 108 Cases with Bone Langerhans Cell Histiocytosis in Children

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 clinicopathological, pathomorphological, immunochemical characteristics, different diagnosis and the prognosis of bone langerhans cell histiocytosis（LCH） in children. Methods Review was done in 108 cases of children＇s bone LCH, using the envision way of immunohistochemistry dyeing on 40 cases, to reclassify them according to the WHO separate criteria made in 2000, then followed them up. Results Seventy - nine cases were situated in solitary bone 73.14% （79/108） , 27 cases were situated in bones（ with or without skin involved） 25% （27/108）, 2 cases involved multi- organs （bone, liver, spleen, and so on） 1.9% （2/108）. The five - year survival rate in the first 2 situations was 100% ,while 2 cases involving multi - organs were dead in the following 25 months. Forty cases of them were immunostained, and the Langerhans cells expressed as CDla （ ＋ ） 100% （40/40）, Vim （ ＋ ）90% （36/40）, S100 （ ＋ ）90% （36/40）, CD6s（ ＋ ）67.5% （27/40）, Lys （ ＋ ）40% （16/40）, MAC387 （ ＋ ）30% （12/40）, CK （ - ）, EMA （ - ）. Conclusions Bone LCH in children is a kind of agnogenic neoplastic hyperplasia disease. It has specially clinicopatbological and immunohistochemicaI characteristics. Its prognosis is related to both the range of bone involvement and histological classification, most of which has a good prognosis.