BACKGROUND Langerhans cell histiocytosis (LCH) is a rare disease of unknown aetiology. While it may affect any organ of the body, few cases of solitary lung involvement are published in the literature. Here, we report...BACKGROUND Langerhans cell histiocytosis (LCH) is a rare disease of unknown aetiology. While it may affect any organ of the body, few cases of solitary lung involvement are published in the literature. Here, we report a rare case of pulmonary LCH (PLCH) in an adult. CASE SUMMARY A 52-year-old male presented to hospital in July 2018 with complaints of progressively worsening cough with sputum, breathlessness, easy fatigability, and loss of appetite since 2016, and a 32-year history of heavy cigarette smoking (average 30 cigarettes/d). Physical examination showed only weakened breathing sounds and wheezing during lung auscultation. Chest computed tomography (CT) showed irregular micronodules and multiple thin-walled small holes. Respiratory function tests showed a slight decrease. Ultrasonic cardiogram showed mild tricuspid regurgitation and no pulmonary hypertension. Fibreoptic bronchoscopy was performed with transbronchial biopsies from the basal segment of right lower lobe. LCH was confirmed by immunohistochemistry. The final diagnosis was PLCH without extra-pulmonary involvement. We suggested smoking cessation treatment. A 3-mo follow-up chest CT scan showed clear absorption of the nodule and thin-walled small holes. The symptoms of cough and phlegm had improved markedly and appetite had improved. There was no obvious dyspnoea. CONCLUSION Imaging manifestations of nodules, cavitating nodules, and thick-walled or thinwalled cysts prompted suspicion of PLCH and lung biopsy for diagnosis.展开更多
BACKGROUND Pulmonary Langerhans cell histiocytosis(PLCH)is a rare cystic lung disease usually affecting young adults.It is predicted that PLCH is a lung tumor precursor associated with dysfunction of the myeloid dendr...BACKGROUND Pulmonary Langerhans cell histiocytosis(PLCH)is a rare cystic lung disease usually affecting young adults.It is predicted that PLCH is a lung tumor precursor associated with dysfunction of the myeloid dendritic cells in the lung.CASE SUMMARY A 70-year-old male patient presented with chronic cough and sputum.He had symptoms for 5 years and described shortness of breath on exertion for the previous 3 years.He had a 60 packs/year smoking history.Computerized tomography of the thorax revealed an 11-mm nodule in the right lung lower lobe superior segment and a 7-mm nodule in the right lung lower lobe poster basal segment.Those two nodules were resected by means of right thoracoscopic surgery.Pathological evaluation revealed a squamous cell carcinoma and PLCH.CONCLUSION Coexistent squamous cell carcinoma and PLCH suggest possible association between PLCH and lung cancer.展开更多
BACKGROUND Pulmonary Langerhans cell histiocytosis(PLCH)is a relatively rare type of lung disease,common in middle-aged smoking men.It is characterized by proliferation and infiltration of Langerhans cells,and the for...BACKGROUND Pulmonary Langerhans cell histiocytosis(PLCH)is a relatively rare type of lung disease,common in middle-aged smoking men.It is characterized by proliferation and infiltration of Langerhans cells,and the formation of multiple parabronchial mesenchymal nodules in lung tissue,and may lead to organ dysfunction.There are no typical symptoms and signs,and it is easily misdiagnosed or missed,and therefore deserves clinical attention and further discussion.CASE SUMMARY We describe the case of a nonsmoking 46-year-old man with PLCH diagnosed based on clinical manifestations of fever and dry cough,with a history of hypothyroidism and diabetes insipidus for 9 years.Computed tomography(CT)-and CT-guided puncture examinations revealed no abnormalities,and he ultimately underwent thoracoscopic biopsy to confirm the diagnosis.The pathological diagnosis was PLCH.Thyroid function was maintained by medication.Pituitary magnetic resonance imaging showed that the pituitary stalk had become thinner.CONCLUSION LCH often involves multiple systems.Moreover,the pathogenesis is not clear,clinical manifestations lack specificity,and diagnosis requires special attention.Diagnosis of PLCH can significantly benefit from comprehensive multidisciplinary analysis.展开更多
Objectives Langerhans’ Cell histiocytosis (LCH) is a rare disease, which remains poorly understood and whose cellular origin remains unknown. To increase understanding of temporal bone LCH, it is necessary to study r...Objectives Langerhans’ Cell histiocytosis (LCH) is a rare disease, which remains poorly understood and whose cellular origin remains unknown. To increase understanding of temporal bone LCH, it is necessary to study recent advances in the diagnosis and treatment of this disease. Methods The long term(5 to 30 years) results of 21 temporal bone LCH cases treated between 1973 and 2003 were reviewed. Surgery, radiotherapy, pharmacologic therapy or a combination of these treatments were employed in these cases. Results Eighteen patients were cured(18/21, 85%). Six patients developed residual diabetes insipidus (DI) and dwarfism (28%). Three patients died(14%). Conclusions The Alessi classification system for LCH based on the extent of disease accurately predicts prognosis and is a useful guide in selecting treatment methodologies. X-ray, computed tomography and magnetic resonance imaging have proved useful in defining the extent of osseous and soft tissue diseases. Diagnosis of LCH is based on clinical presentations, radiographic findings and histopathological results. Surgery and radiotherapy are the main treatment modalities. Pharmacologic therapy should be used in patients with aggressive, disseminate, and refractory lesions. LCH has a predilection for children and prognosis depends on age and extent of vital organ involvement.展开更多
Clinical categories of Langerhans cell histiocytosis （LCH） include single and multi-system disease. Pulmonary LCH is rare, which is an unusual interstitial lung disease with the characteristics of monoclonal prolife...Clinical categories of Langerhans cell histiocytosis （LCH） include single and multi-system disease. Pulmonary LCH is rare, which is an unusual interstitial lung disease with the characteristics of monoclonal proliferation and infiltration of Langerhans＇ cells to organs. We report our experience of a rare LCH case of multiple organs such as pulmonary and liver as the main clinical manifestation. The patient was treated with chemotherapy which included prednisone, vinblastine, methotrexate and 6-mercaptopurine for 52 weeks and follow up all along. The patient has a favorable clinical outcome.展开更多
目的探讨肺朗格汉斯细胞组织细胞增生症(pulmonary Langerhans cell histiocytosis,PLCH)的临床表现、影像学、病理及预后特征,以提高临床医生对该病的认识。方法回顾性分析广州医科大学附属第一医院2009年9月至2019年8月收治住院的PLC...目的探讨肺朗格汉斯细胞组织细胞增生症(pulmonary Langerhans cell histiocytosis,PLCH)的临床表现、影像学、病理及预后特征,以提高临床医生对该病的认识。方法回顾性分析广州医科大学附属第一医院2009年9月至2019年8月收治住院的PLCH患者36例,对其临床资料进行分析。根据Muller评分法进行评分,对结节状病灶、囊状病变及肺间质性病变的HRCT评分进行比较。根据患者第1次行气管镜肺活检(transbronchial lung biopsy,TBLB)是否获得阳性结果将患者分为两组:阳性组(11例)和阴性组(8例);根据确诊PLCH的肺病理中浸润组织的嗜酸粒细胞的量分为两组:较多嗜酸粒细胞浸润组(18例)和较少嗜酸粒细胞浸润组(13例);根据患者的预后分为:存活组(32例)和死亡组(4例)。对两组间的临床特征进行比较。结果36例PLCH患者发病中位年龄27.50(19.50,36.75)岁,55.56%(20/36)的患者出现过自发性气胸,36例PLCH患者的HRCT总评分平均数为(6.71±2.84)分;结节状病灶、囊状病变及肺间质性病变的HRCT评分中位数分别为2.67(0.67,4.33)分、3.67(1.75,4.33)分和0.00(0.00,1.92)分,且三者评分的差异具有统计学意义(χ^(2)=18.000,P<0.001)。囊状病变HRCT评分与第1秒用力呼气容积占预计值的百分比(the percentage of predicted value of forced expiratory volume in 1second,FEV1%pred)及第1秒用力呼气容积/用力肺活量(forced expiratory volume in 1second/forced vital capacity,FEV1/FVC)呈负相关(分别为:rs=-0.527,P=0.008;rs=-0.440,P=0.032)。19例患者进行TBLB,57.89%(11/19)第1次进行TBLB阳性组单次呼吸法肺一氧化碳弥散量占预计值的百分比(the percentage of predicted value of diffusion capacity for carbon monoxide of lung-single breath method,DLCO SB%pred)平均值为(75.19±11.91)%,明显优于阴性组[(55.43±17.10)%],差异有统计学意义(t=2.449,P=0.032);阳性组肺间质性病变HRCT评分均为0.00分,明显低于阴性组[4.50(0.00,4.92)],差异有统计学意义(Z=-2.932,P=0.020)。确诊的PLCH肺病理组织中,有较多嗜酸粒细胞浸润组有61.11%(11/18)发现结节病灶,明显多于较少嗜酸粒细胞浸润组(15.38%)(Fisher精确概率法,P=0.025)。4例(11.11%,4/36)患者死亡,死亡组(100.00%)为多系统受累的PLCH,明显多于存活组(31.25%,10/32),差异有统计学意义(Fisher精确概率法,P=0.017)。结论对于气胸为主要临床表现、囊状及结节状病变为主要影像学改变和/或伴有多系统受累的患者需考虑PLCH的可能;对于怀疑PLCH患者如弥散功能较好、HRCT中无间质性病变的表现,建议可尝试行TBLB确诊;多系统受累为预后不利因素。展开更多
目的探讨肺朗格汉斯细胞组织细胞增生症(PLCH)的发病机制及治疗进展。方法检索PubMed及Web of Science等数据库中相关文献,检索时限为自建库起至2021年2月,分析PLCH的发病机制和治疗进展。结果与结论目前认为,PLCH发病与吸烟及丝裂原活...目的探讨肺朗格汉斯细胞组织细胞增生症(PLCH)的发病机制及治疗进展。方法检索PubMed及Web of Science等数据库中相关文献,检索时限为自建库起至2021年2月,分析PLCH的发病机制和治疗进展。结果与结论目前认为,PLCH发病与吸烟及丝裂原活化蛋白激酶(MAPK)通路的基因突变相关。戒烟在PLCH的治疗中发挥着重要作用。药物治疗方面,除传统使用的皮质类固醇和化疗药物外,MAPK通路抑制剂为该病的治疗提供了新的思路和更好的选择。展开更多
文摘BACKGROUND Langerhans cell histiocytosis (LCH) is a rare disease of unknown aetiology. While it may affect any organ of the body, few cases of solitary lung involvement are published in the literature. Here, we report a rare case of pulmonary LCH (PLCH) in an adult. CASE SUMMARY A 52-year-old male presented to hospital in July 2018 with complaints of progressively worsening cough with sputum, breathlessness, easy fatigability, and loss of appetite since 2016, and a 32-year history of heavy cigarette smoking (average 30 cigarettes/d). Physical examination showed only weakened breathing sounds and wheezing during lung auscultation. Chest computed tomography (CT) showed irregular micronodules and multiple thin-walled small holes. Respiratory function tests showed a slight decrease. Ultrasonic cardiogram showed mild tricuspid regurgitation and no pulmonary hypertension. Fibreoptic bronchoscopy was performed with transbronchial biopsies from the basal segment of right lower lobe. LCH was confirmed by immunohistochemistry. The final diagnosis was PLCH without extra-pulmonary involvement. We suggested smoking cessation treatment. A 3-mo follow-up chest CT scan showed clear absorption of the nodule and thin-walled small holes. The symptoms of cough and phlegm had improved markedly and appetite had improved. There was no obvious dyspnoea. CONCLUSION Imaging manifestations of nodules, cavitating nodules, and thick-walled or thinwalled cysts prompted suspicion of PLCH and lung biopsy for diagnosis.
文摘BACKGROUND Pulmonary Langerhans cell histiocytosis(PLCH)is a rare cystic lung disease usually affecting young adults.It is predicted that PLCH is a lung tumor precursor associated with dysfunction of the myeloid dendritic cells in the lung.CASE SUMMARY A 70-year-old male patient presented with chronic cough and sputum.He had symptoms for 5 years and described shortness of breath on exertion for the previous 3 years.He had a 60 packs/year smoking history.Computerized tomography of the thorax revealed an 11-mm nodule in the right lung lower lobe superior segment and a 7-mm nodule in the right lung lower lobe poster basal segment.Those two nodules were resected by means of right thoracoscopic surgery.Pathological evaluation revealed a squamous cell carcinoma and PLCH.CONCLUSION Coexistent squamous cell carcinoma and PLCH suggest possible association between PLCH and lung cancer.
文摘BACKGROUND Pulmonary Langerhans cell histiocytosis(PLCH)is a relatively rare type of lung disease,common in middle-aged smoking men.It is characterized by proliferation and infiltration of Langerhans cells,and the formation of multiple parabronchial mesenchymal nodules in lung tissue,and may lead to organ dysfunction.There are no typical symptoms and signs,and it is easily misdiagnosed or missed,and therefore deserves clinical attention and further discussion.CASE SUMMARY We describe the case of a nonsmoking 46-year-old man with PLCH diagnosed based on clinical manifestations of fever and dry cough,with a history of hypothyroidism and diabetes insipidus for 9 years.Computed tomography(CT)-and CT-guided puncture examinations revealed no abnormalities,and he ultimately underwent thoracoscopic biopsy to confirm the diagnosis.The pathological diagnosis was PLCH.Thyroid function was maintained by medication.Pituitary magnetic resonance imaging showed that the pituitary stalk had become thinner.CONCLUSION LCH often involves multiple systems.Moreover,the pathogenesis is not clear,clinical manifestations lack specificity,and diagnosis requires special attention.Diagnosis of PLCH can significantly benefit from comprehensive multidisciplinary analysis.
文摘Objectives Langerhans’ Cell histiocytosis (LCH) is a rare disease, which remains poorly understood and whose cellular origin remains unknown. To increase understanding of temporal bone LCH, it is necessary to study recent advances in the diagnosis and treatment of this disease. Methods The long term(5 to 30 years) results of 21 temporal bone LCH cases treated between 1973 and 2003 were reviewed. Surgery, radiotherapy, pharmacologic therapy or a combination of these treatments were employed in these cases. Results Eighteen patients were cured(18/21, 85%). Six patients developed residual diabetes insipidus (DI) and dwarfism (28%). Three patients died(14%). Conclusions The Alessi classification system for LCH based on the extent of disease accurately predicts prognosis and is a useful guide in selecting treatment methodologies. X-ray, computed tomography and magnetic resonance imaging have proved useful in defining the extent of osseous and soft tissue diseases. Diagnosis of LCH is based on clinical presentations, radiographic findings and histopathological results. Surgery and radiotherapy are the main treatment modalities. Pharmacologic therapy should be used in patients with aggressive, disseminate, and refractory lesions. LCH has a predilection for children and prognosis depends on age and extent of vital organ involvement.
文摘Clinical categories of Langerhans cell histiocytosis （LCH） include single and multi-system disease. Pulmonary LCH is rare, which is an unusual interstitial lung disease with the characteristics of monoclonal proliferation and infiltration of Langerhans＇ cells to organs. We report our experience of a rare LCH case of multiple organs such as pulmonary and liver as the main clinical manifestation. The patient was treated with chemotherapy which included prednisone, vinblastine, methotrexate and 6-mercaptopurine for 52 weeks and follow up all along. The patient has a favorable clinical outcome.
文摘目的探讨肺朗格汉斯细胞组织细胞增生症(pulmonary Langerhans cell histiocytosis,PLCH)的临床表现、影像学、病理及预后特征,以提高临床医生对该病的认识。方法回顾性分析广州医科大学附属第一医院2009年9月至2019年8月收治住院的PLCH患者36例,对其临床资料进行分析。根据Muller评分法进行评分,对结节状病灶、囊状病变及肺间质性病变的HRCT评分进行比较。根据患者第1次行气管镜肺活检(transbronchial lung biopsy,TBLB)是否获得阳性结果将患者分为两组:阳性组(11例)和阴性组(8例);根据确诊PLCH的肺病理中浸润组织的嗜酸粒细胞的量分为两组:较多嗜酸粒细胞浸润组(18例)和较少嗜酸粒细胞浸润组(13例);根据患者的预后分为:存活组(32例)和死亡组(4例)。对两组间的临床特征进行比较。结果36例PLCH患者发病中位年龄27.50(19.50,36.75)岁,55.56%(20/36)的患者出现过自发性气胸,36例PLCH患者的HRCT总评分平均数为(6.71±2.84)分;结节状病灶、囊状病变及肺间质性病变的HRCT评分中位数分别为2.67(0.67,4.33)分、3.67(1.75,4.33)分和0.00(0.00,1.92)分,且三者评分的差异具有统计学意义(χ^(2)=18.000,P<0.001)。囊状病变HRCT评分与第1秒用力呼气容积占预计值的百分比(the percentage of predicted value of forced expiratory volume in 1second,FEV1%pred)及第1秒用力呼气容积/用力肺活量(forced expiratory volume in 1second/forced vital capacity,FEV1/FVC)呈负相关(分别为:rs=-0.527,P=0.008;rs=-0.440,P=0.032)。19例患者进行TBLB,57.89%(11/19)第1次进行TBLB阳性组单次呼吸法肺一氧化碳弥散量占预计值的百分比(the percentage of predicted value of diffusion capacity for carbon monoxide of lung-single breath method,DLCO SB%pred)平均值为(75.19±11.91)%,明显优于阴性组[(55.43±17.10)%],差异有统计学意义(t=2.449,P=0.032);阳性组肺间质性病变HRCT评分均为0.00分,明显低于阴性组[4.50(0.00,4.92)],差异有统计学意义(Z=-2.932,P=0.020)。确诊的PLCH肺病理组织中,有较多嗜酸粒细胞浸润组有61.11%(11/18)发现结节病灶,明显多于较少嗜酸粒细胞浸润组(15.38%)(Fisher精确概率法,P=0.025)。4例(11.11%,4/36)患者死亡,死亡组(100.00%)为多系统受累的PLCH,明显多于存活组(31.25%,10/32),差异有统计学意义(Fisher精确概率法,P=0.017)。结论对于气胸为主要临床表现、囊状及结节状病变为主要影像学改变和/或伴有多系统受累的患者需考虑PLCH的可能;对于怀疑PLCH患者如弥散功能较好、HRCT中无间质性病变的表现,建议可尝试行TBLB确诊;多系统受累为预后不利因素。
文摘目的探讨肺朗格汉斯细胞组织细胞增生症(PLCH)的发病机制及治疗进展。方法检索PubMed及Web of Science等数据库中相关文献,检索时限为自建库起至2021年2月,分析PLCH的发病机制和治疗进展。结果与结论目前认为,PLCH发病与吸烟及丝裂原活化蛋白激酶(MAPK)通路的基因突变相关。戒烟在PLCH的治疗中发挥着重要作用。药物治疗方面,除传统使用的皮质类固醇和化疗药物外,MAPK通路抑制剂为该病的治疗提供了新的思路和更好的选择。
