小儿郎格罕细胞组织细胞增生症八例误诊分析

Analysis misdiagnosis reason of Langerhans cell histocytosis 8 cases in children

目的郎格罕细胞组织细胞增生症（Langerhans cell histiocytosis，下称LCH）较少见，为减少误诊病例的发生，将8例LCH病例的误诊原因进行分析。方法用回顾性分析法对8例LCH病例的误诊原因进行分析。结果8例IJCH病儿中，男6例、女2例；年龄10个月-8岁；主要症状分别为发热7例、咳嗽5例，骨痛、多饮多尿各2例；鼻衄、黄疸、腹泻各1例；病程1个月-半年；主要体征分别为头部肿物、皮疹各5例；淋巴结肿大，肺部湿罗音各4例，肝脾肿大3例；主要实验室检查6例病理片找到大量LC及X线、CT示颅骨缺损；贫血3例，找到大量嗜酸细胞及异型巨细胞1例；误诊的疾病分别为尿崩症、肺炎各2例，支气管炎、头皮疖肿、关节炎、菌痢各1例，诊断不明1例；最后确诊勒雪综合征（LSD）、韩薛柯氏病（HSCD）各3例，骨嗜酸性肉牙肿（EG）、2例，除2例失访、1例仍在随访外，其余5例均在2个月内死亡。本病的常见误诊原因有：1．对本病的特殊表现认识不足且询问病史与检查马虎，失去重要线索；2．合并感染，掩盖了原发病；3．未能结合临床正确分析辅助检查。结论要重视LCH的症状、体征和实验室检查，综合分析从而得出正确的诊断，以免误诊。

Objective ： We have seen LCH very little, in order to reduce missed cases take place, we analysis reason of misdiagnosis in 8 cases. Methods- We used look back analysis method proceed reason of misdiagnosis. Results： Misdiagnosis asdiabetes insipidus were 2 cases , pneumonia were 2 cases , bronchitis , skin boil , arthritis , dysentery ＆ unknown cause of diagnosis was 1 case every . Diagnosis LSD ＆ HSCD were 3 cases every , EG were 2 cases . Common cause of misdiagnosis as follows ： 1. As for special manifest of LCH understand insufficient. 2. Be complicated by infection cover up the LCH. 3. Without correct analysis result of sup- plementary check. Conclusion： Must pay attention to symptom , sign ＆ result of supplementary check , comprehensive analysis various circumstance , obtain thus we can right diagnosis , in order to avoid misdiagnosis.