成人自发性气胸合并孤立性肺朗格汉斯细胞组织细胞增多症一例：罕见疾病的常见表现
Spontaneous pneumothorax with isolated pulmonary Langerhans cell histiocytosis in an adult case: A common manifestation of rare disease.
作者信息
Samareh Fekri Mitra, Salajegheh Faranak, Nakhaie Mohsen, Rezaei Zadeh Rukerd Mohammad
机构信息
Cardiovascular Research Center Institute of Basic and Clinical Physiology Sciences, Kerman University of Medical Sciences Kerman Iran.
Clinical Research Development Unit, School of Medicine Afzalipour Hospital, Kerman University of Medical Sciences Kerman Iran.
出版信息
Clin Case Rep. 2023 Jun 17;11(6):e7567. doi: 10.1002/ccr3.7567. eCollection 2023 Jun.
KEY CLINICAL MESSAGE
Pulmonary Langerhans cell histiocytosis should be evaluated even in adults with no previous medical history and no history of cigarette smoking who have spontaneous pneumothorax and evidence of multiple lung cystic lesions, and other organs should also be checked for multi organ Langerhans cell histiocytosis involvement.
ABSTRACT
A 30-year-old man presented with sudden chest pain and evidence of multiple cystic lesions in both upper and lower lobes of lungs, as well as left-sided pneumothorax in high resolution computed tomography. In lung samples, hematoxylin and eosin- stained sections and IHC for CD1a, S100, and BRAF V600 were positive. The patient was diagnosed with isolated pulmonary Langerhans cell histiocytosis and was treated accordingly.
关键临床信息
即使是既往无病史且无吸烟史的成年人，若出现自发性气胸且有多个肺囊性病变的证据，也应评估是否为肺朗格汉斯细胞组织细胞增多症，同时还应检查其他器官是否存在多器官朗格汉斯细胞组织细胞增多症累及情况。
摘要
一名30岁男性因突发胸痛就诊，高分辨率计算机断层扫描显示双肺上叶和下叶有多个囊性病变以及左侧气胸。在肺组织样本中，苏木精-伊红染色切片以及CD1a、S100和BRAF V600的免疫组化检查均呈阳性。该患者被诊断为孤立性肺朗格汉斯细胞组织细胞增多症并接受了相应治疗。
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