郎格罕细胞组织细胞增生症的临床特征与诊断

背景与目的：郎格罕细胞组织细胞增生症（Langerhams cell hyperplasia,LCH）发病逐步增多,临床表现多样化而易误诊。本研究在于了解其临床特征,有利于早期诊断与治疗,评价预后。方法：将近10年来中山大学附属第一医院诊断的44例LCH作回顾性分析。结果：LCH主要发生于小儿,占72．7％（32／44）。成人占27．3％（12／44）,2岁以下小儿占小儿发病的46．9％（15／32）。侵犯的组织器官常见有骨骼20例（45．5％）,淋巴结20例（45．5％）,皮肤16例（36．4％）,肝脾16例（36．4％）,肺14例（31．8％）,骨髓6例（13．6％）,耳4例（9．1％）,眼2例（4．5％）,垂体2例,胸腺1例。其中1个脏器受损10例,2个脏器受损11例,3个脏器受损9例,4个脏器受损7例,5个以上脏器受损7例,肝、肺、骨髓等多功能受损中,小儿22例,成人4例．表现多样化。误诊26例（59．1％）,其中误诊皮肤病6例,其它血液病7例,骨骼及骨髓疾病7例、肺结核2例、尿崩症2例、耳及眼疾病各1例,小儿病例并发支气管肺炎6例,致败血症4例;死f6例,其中2岁以下小儿5例。结论：LCH临床易于误诊,2岁以下小儿多脏器功能衰竭及并发支气管肺炎常见,且死亡率高。作相关组织细胞病理检查有助鉴别,有利早期诊断,早治疗,防止严重并发症。

Clinical features and diagnosis of langerhans cell hyperplasia

BACKGROUND & OBJECTIVE: The incidence rate oflangerhans cell hyperplasia (LCH) is increasing. Its clinical manifestation isso diversified that it is easy to be misdiagnosed. This study was to analyzeits clinical features for the sake of early diagnosis and treatment. METHODS :Clinical data of 44 LCH patients, treated from Jan. 1990 to Dec. 2003 in theFirst Affiliated Hospital of Sun Yat-sen University, were retrospectivelyanalyzed. RESULTS: Of the 44 LCH patients, 32 (72.7% ) were children,12 (27.3%) were adults. Of the 32 children patients, 15 (46.9%) were lessthan 2 years old. The involved organs were bone (20 /44, 45.5% ) , lymphnodes (20 /44, 45.5% ) , skin (16 /44, 36.4% ) , liver and spleen (16 /44,36.4%) , lung (14 /44, 31.8%) , bone marrow (6 /44, 13.6%) , ear (4 /44,9.1% ) , eyes (2 /44, 4.5% ) , pituitary gland (2 /44, 4.5% ) , and thymusgland (1 /44, 2.3%). Of the 44 patients, 10 had 1 organ involved, 11 had 2organs involved, 9 had 3 organs involved, 7 had 4 organs involved, 7 hadno less than 5 organs involved; 22 children patients and 4 adult patients hadmultiple organs (liver, lung, bone marrow, and so on) involved; 26( 59.1% ) were misdiagnosed as dermatosis ( 6 cases) , hemopathy ( 7cases) , bone or bone marrow diseases (7 cases) , lung tuberculosis (2cases) , diabetes insipidus (2 cases) , ear or eye disease (2 cases). Sixchildren patients had bronchopneumonia, and 4 of them had septicemia. Sixpatients died with 5 cases below the age of 2. CONCLUSIONS: LCH is easyto be misdiagnosed. Children below 2 years old tend to complicate withmultiple organ failure (MOF) and bronchopneumonia which have high deathrate. Pathology examination is helpful for early diagnosis, treatment, andprevention of severe complications.