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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LCH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。
The purpose of Langerhans cell histiocytosis (Langerhans cell histiocytosis, hereinafter referred to as LCH) is rare, in order to reduce the incidence of misdiagnosed cases of misdiagnosis in 8 cases of LCH cases were analyzed. Methods Retrospective analysis of 8 cases of misdiagnosis of LCH cases were analyzed. Results 8 cases of LCH sick children, 6 males and 2 females; aged 10 months to 8 years; the main symptoms were fever in 7 cases, cough in 5 cases, bone pain, polyhydramnios in 2 cases; epistaxis, Jaundice and diarrhea in each case. The duration of disease ranged from 1 month to 6 months. The main signs were head and rash in 5 cases, lymphadenopathy, 4 cases of pulmonary wet rales and 3 cases of hepatosplenomegaly. The main experiment A large number of LC and X-ray were found in the pathological examination of 6 cases of pathological examination. CT showed skull defects, 3 cases of anemia and 1 case of large numbers of eosinophils and abnormal giant cells. The diseases misdiagnosed were diabetes insipidus, 2 cases of pneumonia, 3 cases of bronchitis 1 case of scleroderma, 1 case of arthritis and bacillary dysentery, 1 case of unknown diagnosis, 3 cases of LSD and 3 cases of HSK, bone eosinophilic meatal instability (EG) , 2 cases, except 2 cases lost to follow-up, 1 case still under follow-up, the other 5 cases all died within 2 months. Common causes of misdiagnosis of this disease are: 1. The lack of awareness of the disease’s special performance and ask the slightest history and check sloppy, lost important clues; 2. Combined infection, covering up the original disease; 3. Failure to correctly analyze the clinical auxiliary examination . Conclusion The importance of LCH symptoms, signs and laboratory tests, a comprehensive analysis to arrive at the correct diagnosis, so as not to misdiagnosis.