36例朗格罕细胞组织细胞增生症的临床与病理分析  

Clinical and pathologic analysis of Langerhans cell histiocytosis

作　　者：刘庆玲[1] 邹继珍[1] 师晓东[1] 刘嵘[1] 李君惠[1] 王天有[1] 

机构地区：[1]北京首都儿科研究所附属儿童医院血液科,100020

出　　处：《中国小儿血液与肿瘤杂志》2008年第5期220-223,共4页Journal of China Pediatric Blood and Cancer

摘　　要：目的分析朗格罕细胞组织细胞增生症(langerhans cell histiocytosis,LCH)传统分型和单系统/多系统分型及Lavin-Osband分级之间的联系,观察LCH病理结果,并对比临床和预后中的不同。方法回顾性调查36例LCH,分析比较其临床、病理及预后。结果36例LCH中14例单系统LCH,Lavin-OsbandⅠ、Ⅱ级,治疗后痊愈或好转12例。22例多系统LCH,多为Ⅲ、Ⅳ级;治疗后未愈或恶化9例,死亡2例。病理检查17/17例CD1a阳性;电镜6/10例找到Birbeck颗粒;12/12例Fascin染色阳性。结论现行分型分级利于直观评估病情、预后。CD1a染色比电镜找Bir-beck颗粒更简便易行。Fascin在LCH诊断中有一定作用,但其价值需大样本量的试验证实。Objective To discuss the relationship and difference between the traditional and new classification and Lavin-Osband grades system on evaluating clinical severity and prognosis, and to analyze the pathologic results of LCH through a retrospective analysis of clinical data, pathologic results and prognosis. Methods 36 LCH cases were investigated retrospectively. The data of clinical teatures, pathologic outcomes and prognosis had been analyzed. Results The 36 cases including 14 unisystem cases, mainly Lavin-Osband grade Ⅰ and Ⅱ cases, 12 of them recovered or improved after therapy. The 22 multisystem cases, mostly grade Ⅲ and Ⅳ, 9 of them had no response to therapy, 2 died. Histological biopsy showed all 17 cases staining for CD la were positive, 6 of 10 cases found Birbeck granules. All 12 cases stained for Fascin were positive. Conclusion The new classification can better predict the prognosis. Pathological examination showed CD1 a was important in the diagnosis of LCH for its convenience comparing to electron microscopy. Fascin was helpful for diagnosing, but the value requires advanced research.

关 键 词：朗格罕细胞组织细胞增生症 诊断 病理 预后 

分 类 号：R725.5[医药卫生—儿科]