摘要
[目的]分析肺朗格罕组织细胞增生症（PLCH）的临床及影像学特点,提高对该疾病的诊治水平.[方法]回顾性分析PLCH患者4例的临床表现及胸部高分辨CT（HRCT）,结合其病理学结果,总结的临床、影像及病理学特点.[结果]4例PLCH患者均为男性,以咳嗽（75%）、活动后气短（50%）及呼吸困难（50%）为最常见临床表现.平均病程48.5个月.4例患者均具有吸烟史;胸部HRCT上均存在双肺气囊腔样病变及结节影,以中上肺分布为主,结节直径1～5 mm;3例患者肺内存在网格影,1例患者出现蜂窝肺;2例患者出现肺外受累,分别为L3椎体侧后缘及椎体附件弥漫性溶骨性骨质破坏和中枢性尿崩症;2例病理学诊断患者活检组织内均见大量朗格罕细胞增生.[结论]PLCH的呼吸系统表现缺乏特异性,但胸部影像学表现具有一定特点,关注肺外表现及吸烟史有助于提高对该疾病的诊断及鉴别诊断.
[Obiective] To analyze the clinical and radiological characteristics of pulmonary Langerhans ceil histiocytosis（PLCH） in order to improve the diagnosis and treatment of the disease. [Methods] Clinical mani-festation, chest high resolution computerized tomography（HRCT） and pathological results of 4 PLCH patients were analyzed retrospectively. Clinical, radiological and pathological characteristics of PLCH were summa- rized. [Results] All 4 PLCH patients were male. The commonest clinical manifestations were cough（75%）, exertional dyspnea（50%） and dyspnea（50%）. The average courses of PLCH were 48.5 months. All patients had smoking history. Chest HRCT showed bilateral and diffusive cystic and nodular changes with the predomi-
nance distribution at middle and upper lung fields and the diameter of the nodules was lmm-5mm. Reticular changes were found in 3 cases and honeycomb changes were seen in 1 case. Two cases of extra-pulmonary in-volvement were manifested as diffusive osteolytic changes in posterior third lumbar vertebrae and appendix of vertebra and central diabetes insipidus. Massive Langerhans cell proliferation was found in 2 patients diagnosed by pathological results. [Conclusion] The respiratory manifestations of PLCH are unspecific. But the radio-logical changes are characteristic to some degree. Extra-pulmonary manifestation and the history of smoking history are helpful for improving the diagnosis and differential diagnosis of PLCH.
出处
《医学临床研究》
CAS
2012年第5期801-804,共4页
Journal of Clinical Research
