郎格罕组织细胞增生症五例临床分析

Clinical and pathological features of 5 adult patients with Langerhans cell histiocytosis

目的 提高对郎格罕组织细胞增生症 (LCH)的认识。方法 回顾分析我院近 10年来确诊的 5例LCH成年患者的临床、影像和病理资料。结果 男 3例 ,女 2例 ,平均年龄 36 8岁。所有病例均表现有肺、骨骼、中枢神经系统、皮肤、肝、脾、淋巴结等多器官病变。LCH肺部病变的典型放射影像学表现为双肺弥漫结节影 ,间质纤维化伴多发囊疱形成。有 3例患者行骨X线检查 ,可见低密度骨质破坏灶。全部患者的活检标本均可见异常的郎格汉斯细胞浸润。对 5例患者均予反复全身化疗(激素 +蒽环类细胞毒药物 ) ,化疗对肝、脾、淋巴结、皮肤病变的疗效较好 ,而对肺部病变、中枢性尿崩症、骨损害的疗效较差。结论 LCH可在任何年龄发病 ,对有尿崩症、特征性骨质破坏和肺部病变的患者应警惕此病 ,并及时行病灶部位的病理学检查 ,确诊后予放疗。

Objective To improve the identification of Langerhans cell histiocytosis (LCH). Methods 5 adult cases of LCH treated in our hospital since 1990 were studied, the clinical data and pathological findings were analyzed. Results The sex ratio of male to female was 3 to 2, the mean age in this series of 5 patients was 36.8 years . All patients presented with multifocal or disseminated form of the disease .The involved organs included lung, skeleton, central nervous system, skin, liver, spleen and lymph node. The radiographic presentation of lung involvement includes diffuse micronodular densities(1 case),interstitial fibrosis with cyst formation(2 cases), limited infiltration in unilateral lung field(1 case)and isolated mass(1 case).Sharply demarcated bony rarefaction in radiogram was found in 3 cases. The presence of specific Langerhans cell in the focal lesion, which was the pathological basis for the definitive diagnosis of LCH, was detected in all patients. Systemic chemotherapy with corticosteroid and anthracycline based regimen was given to 5 patients and it caused retraction of the enlarged liver and spleen in 1 case ,amelioration of lymph nodes and skin involvement in 2 cases and diminution of enlarged thyroid gland in 1 case ,while it had less benefit in lung and bone involvement or to diabetes insipidus. Conclusions LCH can be observed in young adults or middle-aged subjects .It should be suspected in patients who presented with diabetes insipidus, characteristic bony lesion and pulmonary involvement. The definitive diagnosis should be made pathologically in time. The proper treatment with low-dose radiotherapy and/or systemic chemotherapy should be taken based on the number and the function of systems involved.