[成人朗格汉斯细胞组织细胞增多症与硬化性胆管炎]
[Langerhans cell histiocytosis and sclerosing cholangitis in adults].
作者信息
Gey T, Bergoin C, Just N, Paupard T, Cazals-Hatem D, Xuan K H, Tavernier J-Y, Wallaert B
机构信息
Clinique des Maladies respiratoires, Hôpital Albert Calmette, CHRU Lille, boulevard de Professeur Jules Leclercq, 59037 Lille Cedex, France.
出版信息
Rev Mal Respir. 2004 Nov;21(5 Pt 1):997-1000. doi: 10.1016/s0761-8425(04)71483-7.
INTRODUCTION
Langerhans cell histiocytosis and sclerosing cholangitis are two rare diseases that are frequently linked in children, but very rarely so in adults.
CASE REPORT
A 40 year old woman with a 17 year history of Langerhans cell histiocytosis with chronic respiratory failure and diabetes insipidus presented with cholestatic jaundice whilst being assessed for lung transplantation. Pathological examination demonstrated sclerosing cholangitis. No Langerhans histiocytosis lesions were found in the liver or the biliary tract. Plans for pulmonary and hepatic transplantation were abandoned after cerebral involvement was detected, and the patient died of acute hepatic failure.
CONCLUSION
This case underlines the need to monitor liver function in adult patients with disseminated Langerhans histiocytosis associated in adults, as coexisting sclerosing cholangitis is associated with a poor prognosis.
引言
朗格汉斯细胞组织细胞增多症和硬化性胆管炎是两种罕见疾病，在儿童中常有关联，但在成人中极为罕见。
病例报告
一名40岁女性，有17年朗格汉斯细胞组织细胞增多症病史，伴有慢性呼吸衰竭和尿崩症，在接受肺移植评估时出现胆汁淤积性黄疸。病理检查显示为硬化性胆管炎。肝脏和胆道未发现朗格汉斯组织细胞增多症病变。在检测到脑部受累后，放弃了肺和肝移植计划，患者死于急性肝衰竭。
结论
该病例强调了对成人播散性朗格汉斯组织细胞增多症患者监测肝功能的必要性，因为并存的硬化性胆管炎与预后不良相关。
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