摘要
目的 了解朗格汉斯细胞组织细胞增生症 (LCH)肺损害的临床情况。方法 回顾性分析近 10年诊断的 4 4例LCH的临床资料。结果 4 4例LCH中肺受累 14例 (占 33% ) ,其中小儿10例 ,占小儿LCH发病的 31% ;成人 4例 ,占成人LCH发病的 33%。无呼吸道症状 5例 ,咳嗽、咳痰8例 ,其中伴气促 2例。影像学表现主要为肺间质损害 ,表现为纹理增多、增粗、紊乱、蜂窝状、网点状、小结节状改变等 ,亦可见肺气肿、胸腔积液、胸膜增厚 ,以及肺门纵隔淋巴结增大。小儿合并支气管肺炎 6例 ,曾误诊为原发病 4例 ,其中 2岁以下 5例 ,致金黄色葡萄球菌败血症 2例 ,终因多脏器功能衰竭死亡 ;误为肺结核 2例及乳糜胸 1例。化疗后 ,症状及肺部病变可明显改善。结论 肺是LCH常累及脏器 ,有影像学改变不一定出现呼吸道症状 ,尤其 2岁以下小儿易合并支气管肺炎、败血症及多脏器功能衰竭。LCH肺损害无临床特异性而易误诊 ,胸部CT有利于早期发现肺损害病变。及时诊断、化疗 。
Objective To understand the clinical manifestations of lung injury in Langerhans cell histocytosis (LCH).Methods A clinical retrospective analysis on 44 cases diagnosed as LCH in the past decade were performed.Results 14 of 44 cases with LCH were found with lung injury(32%).Child patients with lung injury made up 31% of all child patients with LCH and adult patients with lung injury made up 33% of all adult patients with LCH.5 cases were found without respiratory symptoms.Cough and expectoration were found in 6 cases Breathlessness was found in 2 cases.Changes in X-ray examination were as follow:interstitial changes,rougher and more prominent venations,alveolate,reticular or nodular lesions,etc.Emphysema,pleural effusion,pleural thickness,and enlargement of portal and mediastinal lymph nodes could also be found.4 cases were misdiagnosed as primary bronchial pneumonia,while 2 as tuberculosis and l as chylothorax.6 child patients were complicated with bronchial pneumonia with 5 of them under 2 year old.2 child suffered from MRSA septicemia and died of multiple system organ failure.Obvious improvements of symptoms and lung lesions occurred after chemotherapy.Conclusions Lung was one of the most predisposing organ to be damaged in LCH.Respiratory symptoms did not always present despite the evidence of X-ray abnormalities Child patiens,especially those under 2 year old,tended to catch bronchial pneumonia,sepicemia and multiple system organ failure.Lung injury in LCH was ofen misdiagnosed due to lack of specific symptoms.CT scan was helpful in early diagnosis of lung injury.Lung lesions may improved if diagnosis and chemotherapy were in time.
出处
《中国呼吸与危重监护杂志》
CAS
2004年第4期226-228,共3页
Chinese Journal of Respiratory and Critical Care Medicine
