| 摘要: |
| 【】 目的 探讨朗格汉斯细胞组织细胞增生症( LCH) 的临床特征、病理学、免疫表型、治疗及预后。方法 回顾性分析2例经病理确诊的成人LCH的临床资料,并结合文献报道,分析其临床、病理特征以及治疗和预后情况。结果 病例1为年轻女性,病程仅1月,临床表现为咳嗽咳痰,肺部CT显示双肺多发大小不等结节,伴薄壁空洞,开胸肺活检及免疫组化提示病变组织S-100( )、CD1a( )、CD68( ),无其他器官受累,经戒烟及激素治疗后病灶基本消失；病例2为老年女性,病程半年,表现为反复皮疹及全身淋巴结肿大,经淋巴结活检及免疫组化确诊为LCH,经COP方案化疗后病情好转,至今无复发。结论 朗格汉斯细胞组织细胞增生症临床罕见,误诊率高,应结合多系统临床表现及组织学特征、免疫表型综合分析。 |
| 关键词: 组织细胞增多症 朗格汉斯细胞 吸烟 组织学 |
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| Langerhans cell histiocytosis: two cases report and review of literature |
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Luo zhuanbo1, Huang xiaoping1, Xu ning1, Li chenwei2, Piao zhenghua3
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1.nignbo No.1 hospital;2.Ningbo No.1 hospital;3.Ningbo clinical pathological diagnosis center
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| Abstract: |
| ［］ Objective To investigate the clinical, pathological, immunophenotypical features, treatment and prognosis of langerhans cell histiocytosis(LCH). Methods The information of two LCH cases who were diagnosed by pathology were reviewed. Results Case one was a young woman with 1 month of course of disease, manifesting with prominent pulmonary involvement: cough and phlegm; CT scan of the chest showed lung nodules with different size and shape and thin wall cavity involving all lobes of both lungs. Open lung biopsy and immunohistochemistry showed S-100( ), CD1a( ), CD68( ), no other organs involved. The lesions disappeared after treatment for smoking cessation and hormone. Case 2 was a old woman with 6 month of course of disease, characterized by skin rashes and systemic lymph node enlargement. Lymph node biopsy was given and immunohistochemical diagnosis of LCH was made. After chemotherapy of COP, the condition improved, without recurrence. Conclusion Langerhans cell histiocytosis is extremely rare. The clinical presentation of LCH is highly variable. |
| Key words: Histiocytosis Langerhans-cell Smoking Histology |