肺结晶体贮积性组织细胞增生症1例  

Pulmonary crystal-storing histiocytosis:a case report

作　　者：陈晓炎[1] 雷正瑶 薄佳琪 王顺利 易祥华[2] Chen Xiaoyan;Lei Zhengyao;Bo Jiaqi;Wang Shunli;Yi Xianghua(Department of Pathology,Ruijin Hospital,Shanghai Jiao Tong University School of Medicine,Shanghai 200025,China;Department of Pathology,Tongji Hospital,Tongji University,Shanghai 200065,China)

机构地区：[1]上海交通大学医学院附属瑞金医院病理科,上海200025 [2]同济大学附属同济医院病理科,上海200065

出　　处：《中华结核和呼吸杂志》2023年第6期595-598,共4页Chinese Journal of Tuberculosis and Respiratory Diseases

基　　金：国家自然科学基金(82170082);上海市卫生局医学重点专项(20134034)。

摘　　要：本文报道1例发生于肺的结晶体贮积性组织细胞增生症(crystal-storing histiocytosis,CSH)。患者体检发现肺部肿物,胸部CT示右肺下叶结节影,边缘毛糙,分叶状,增强后见明显强化,邻近胸膜受牵拉。PET-CT示代谢稍高,考虑恶性病变。行右肺下叶楔形切除术。根据病理形态及免疫组织化学(immunohistochemistry,IHC)诊断为CSH。患者术后随访41个月,未提示病灶复发,未提示其他新发疾病。CSH是一种罕见的非肿瘤性组织细胞增生性疾病,以嗜酸性晶体物质在组织细胞胞质内聚集为特征。该疾病往往与淋巴细胞增生性疾病或浆细胞性疾病相关,诊断后需进行全身检查,并建议长期随诊。A 45-year-old female patient was found to have a nodule in the right lower lobe on physical examination.Chest CT showed the nodule was lobulated measuring 24 mm×23 mm,with obvious enhancement and adjacent pleural traction.As the PET-CT showed increased 18F-FDG uptake suggesting malignancy,the wedge resection of the right lower lobe was performed.Grossly,the mass was adjacent to the pleural area with indistinct boundary.On cut sections,the lesion was solid and tough,with a greyish-pink colour.Microscopically,the lesion had an ill-defined margin,and was composed of spindle and polygonoid histiocytes with rich eosinophilic cytoplasm similar to rhabdoid muscle cells.The cytoplasm of histiocytes was filled with diamond-shaped or club-shaped crystals.Immunohistochemistry(IHC)showed the histiocytes were positive for CD68,κ,λ,IgG,IgM and IgA.The patient had been followed up for 41 months and had shown neither recurrences nor new diseases.CSH is a rare non-neoplastic histiocytic proliferative disease.Pulmonary CSH should be differentiated from multiple diseases.Accurate pathological diagnosis depends on its morphology and immunophenotype.This disease is often related to potential lymphoproliferative or plasma cell disorder.After diagnosis,a systemic examination is required and long-term follow-up is recommended.

关 键 词：组织细胞增生症 右肺下叶 全身检查 非肿瘤性 恶性病变 胸部CT 结节影 分叶状 

分 类 号：R563[医药卫生—呼吸系统]