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
Langerhans cell histiocytosis has long been described as a rare systemic disorder involving the proliferation of Langerhans cells with formation of granuloma. The disease may be localised or diffuse. Typical forms of ...Langerhans cell histiocytosis has long been described as a rare systemic disorder involving the proliferation of Langerhans cells with formation of granuloma. The disease may be localised or diffuse. Typical forms of the disease involving the bone, the skin or the pituitary gland have been well described whereas others, such as thymic histiocytosis, are still poorly understood. Here, we report a case of isolated Langerhans cell histiocytosis of the thymus in an infant with non-specific symptoms. We provide a description of the disease and discuss patient management.展开更多
Background： Pulmonary Langerhans cell histiocytosis （PLCH） is an interstitial primary pulmonary disease, characterized by Langerhans cell proliferation. It is easily misdiagnosed in children. This study aimed to ch...Background： Pulmonary Langerhans cell histiocytosis （PLCH） is an interstitial primary pulmonary disease, characterized by Langerhans cell proliferation. It is easily misdiagnosed in children. This study aimed to characterize the clinical manifestations and features of PLCH by retrospective analysis. Meihods： A retrospective analysis was performed in 117 PLCH patients out of 338 LCH patients who were admitted in our center from November 2006 to October 2013. Variables between two groups were compared by Mann-Whitney U-test and Chi-square test. Kaplan-Meier curves were constructed to compare the survival rates and Cox regression to evaluate the effect of risk factors. Results： The median age of PLCH group was significantly lower than that ofnon-PkCH group （ 18.63 months vs. 43.4 months, P 〈 0.001 ）. All PLCH children had other organ involvement and only 11 cases （9.4%） had respiratory symptoms. The most common radiologic finding was cystic lesions （29 cases, 24.8%）. Pulmonary thnction abnormalities were dominated by obstructive ventilatory dysfunction （63 cases, 82.9%）. The 5-year overall survival （OS） of PECH children was 93.6% ± 2.3% and the event-free survival （EFS） was 55.7% ± 5.2%. Among the 38 cases with progressed or relapsed disease, five cases （13.2%） were due to progression or recurrence of lung damage. The 5-year OS of PECH children with ＂risk organ＂ involvement was significantly lower than those without ＂risk organ＂ involvement （86.0% ± 4.9% vs. 100%,X^2 = 8.793, P= 0.003）. The difference of EFS between two groups was also significant （43.7% ± 7.7% vs. 66.3% ± 6.5%,X^2 = 5.399, P = 0.020）. The ＂＇risk organ＂ involvement had a significant impact on survival （hazard ratio = 1.9, P = 0.039）. Conclusions： PLCH mainly occurs in young children, and only a small percentage of patients have respiratory symptoms. They generally have other organ involvement. Most of PLCH children have a good prognosis and most lung lesions could have improved or stabilized. Management of＂risk organ＂ involvement is the key point to improving EFS.展开更多
目的比较颞骨胆脂瘤和颞骨朗格汉斯组织细胞增生症影像组学特征,为早期确诊提供参考。方法回顾性分析2016年1月至2020年12月在复旦大学附属儿科医院治疗的颞骨胆脂瘤及颞骨朗格汉斯组织细胞增生症患儿的临床资料,利用影像组学特征分析17...目的比较颞骨胆脂瘤和颞骨朗格汉斯组织细胞增生症影像组学特征,为早期确诊提供参考。方法回顾性分析2016年1月至2020年12月在复旦大学附属儿科医院治疗的颞骨胆脂瘤及颞骨朗格汉斯组织细胞增生症患儿的临床资料,利用影像组学特征分析17例(17耳)颞骨胆脂瘤和13例(13耳)颞骨朗格汉斯组织细胞增生症的患儿影像学资料。结果有3种相关系数较大的影像组学稳定特征:相关性的信息量度(Informational Measure of Correlation,IMC),粗度(Coarseness),小区域重点(SmallAreaEmphasis,SAE)。朗格汉斯组织细胞增生症CT体素IMC和胆脂瘤有明显区别。朗格汉斯组织细胞增生症的Coarseness、SAE要明显高于胆脂瘤。结论影像组学特征分析可以作为颞骨病变鉴别诊断的重要辅助手段。IMC,Coarseness,SAE可以作为鉴别颞骨胆脂瘤和颞骨朗格汉斯组织细胞增生症的重要特征,为早期诊断提供重要线索。展开更多
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Langerhans cell histiocytosis has long been described as a rare systemic disorder involving the proliferation of Langerhans cells with formation of granuloma. The disease may be localised or diffuse. Typical forms of the disease involving the bone, the skin or the pituitary gland have been well described whereas others, such as thymic histiocytosis, are still poorly understood. Here, we report a case of isolated Langerhans cell histiocytosis of the thymus in an infant with non-specific symptoms. We provide a description of the disease and discuss patient management.
基金This study was supported by grants from the National Science and Technology Key Projects （No. 2017ZX09304029004）, Beijing Municipal Science and Technology Commission （No. ZI71100001017050）, National Natural Science Foundation of China （No. 81700186）, Scientific Research Common Program of Beijing Municipal Commission of Education （No. KM201710025019）, Pediatric Project of Ai You Foundation （No. AYEK201802）, and Talent Training Project-Fostering Fund of National Natural Science Foundation of Beijing Children＇s Hospital, Capital Medical University （No. GPY201713）.
文摘Background： Pulmonary Langerhans cell histiocytosis （PLCH） is an interstitial primary pulmonary disease, characterized by Langerhans cell proliferation. It is easily misdiagnosed in children. This study aimed to characterize the clinical manifestations and features of PLCH by retrospective analysis. Meihods： A retrospective analysis was performed in 117 PLCH patients out of 338 LCH patients who were admitted in our center from November 2006 to October 2013. Variables between two groups were compared by Mann-Whitney U-test and Chi-square test. Kaplan-Meier curves were constructed to compare the survival rates and Cox regression to evaluate the effect of risk factors. Results： The median age of PLCH group was significantly lower than that ofnon-PkCH group （ 18.63 months vs. 43.4 months, P 〈 0.001 ）. All PLCH children had other organ involvement and only 11 cases （9.4%） had respiratory symptoms. The most common radiologic finding was cystic lesions （29 cases, 24.8%）. Pulmonary thnction abnormalities were dominated by obstructive ventilatory dysfunction （63 cases, 82.9%）. The 5-year overall survival （OS） of PECH children was 93.6% ± 2.3% and the event-free survival （EFS） was 55.7% ± 5.2%. Among the 38 cases with progressed or relapsed disease, five cases （13.2%） were due to progression or recurrence of lung damage. The 5-year OS of PECH children with ＂risk organ＂ involvement was significantly lower than those without ＂risk organ＂ involvement （86.0% ± 4.9% vs. 100%,X^2 = 8.793, P= 0.003）. The difference of EFS between two groups was also significant （43.7% ± 7.7% vs. 66.3% ± 6.5%,X^2 = 5.399, P = 0.020）. The ＂＇risk organ＂ involvement had a significant impact on survival （hazard ratio = 1.9, P = 0.039）. Conclusions： PLCH mainly occurs in young children, and only a small percentage of patients have respiratory symptoms. They generally have other organ involvement. Most of PLCH children have a good prognosis and most lung lesions could have improved or stabilized. Management of＂risk organ＂ involvement is the key point to improving EFS.
文摘目的比较颞骨胆脂瘤和颞骨朗格汉斯组织细胞增生症影像组学特征,为早期确诊提供参考。方法回顾性分析2016年1月至2020年12月在复旦大学附属儿科医院治疗的颞骨胆脂瘤及颞骨朗格汉斯组织细胞增生症患儿的临床资料,利用影像组学特征分析17例(17耳)颞骨胆脂瘤和13例(13耳)颞骨朗格汉斯组织细胞增生症的患儿影像学资料。结果有3种相关系数较大的影像组学稳定特征:相关性的信息量度(Informational Measure of Correlation,IMC),粗度(Coarseness),小区域重点(SmallAreaEmphasis,SAE)。朗格汉斯组织细胞增生症CT体素IMC和胆脂瘤有明显区别。朗格汉斯组织细胞增生症的Coarseness、SAE要明显高于胆脂瘤。结论影像组学特征分析可以作为颞骨病变鉴别诊断的重要辅助手段。IMC,Coarseness,SAE可以作为鉴别颞骨胆脂瘤和颞骨朗格汉斯组织细胞增生症的重要特征,为早期诊断提供重要线索。
