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[Clinical features and diagnosis of langerhans cell hyperplasia]
Kong Zhang1, Hui Zeng, Wei-Qi Chen
1Department of Internal Medicine, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong 510080, P. R. China. zswgzx@sohu.com
Insights
Langerhans cell hyperplasia (LCH) is increasingly diagnosed, often presenting with diverse symptoms leading to misdiagnosis. Early diagnosis through pathology is crucial, especially for young children who face high mortality rates from complications like bronchopneumonia and multiple organ failure.
Area of Science:
- Pediatric Pathology
- Oncology
- Dermatology
Context:
- Langerhans cell hyperplasia (LCH) incidence is rising, presenting with varied clinical manifestations that frequently lead to misdiagnosis.
- Accurate and timely diagnosis of LCH is challenging due to its diverse presentation across multiple organ systems.
- Retrospective analysis of 44 LCH patients treated between 1990 and 2003 provides insights into clinical features and outcomes.
Purpose:
- To analyze the clinical features of Langerhans cell hyperplasia (LCH) to improve early diagnosis and treatment strategies.
- To highlight the diagnostic challenges and common misdiagnoses associated with LCH.
- To identify risk factors and common complications in pediatric LCH cases.
Summary:
- The study analyzed 44 LCH cases, with 72.7% being children, and 46.9% of these children under two years old.
- Commonly involved organs included bone, lymph nodes, skin, liver, spleen, and lungs; 59.1% of patients were initially misdiagnosed.
- Young children (<2 years) with LCH showed a high incidence of bronchopneumonia, sepsis, and multiple organ failure, contributing to a significant mortality rate.
Impact:
- Emphasizes the critical role of pathology examination in the early diagnosis and effective management of LCH.
- Highlights the need for increased clinical awareness of LCH, particularly in pediatric patients presenting with non-specific symptoms.
- Findings underscore the importance of prompt diagnosis and treatment to prevent severe complications and reduce mortality in LCH.
Background & Objective:
The incidence rate of langerhans cell hyperplasia (LCH) is increasing. Its clinical manifestation is so diversified that it is easy to be misdiagnosed. This study was to analyze its clinical features for the sake of early diagnosis and treatment.
Methods:
Clinical data of 44 LCH patients, treated from Jan. 1990 to Dec. 2003 in the First Affiliated Hospital of Sun Yat-sen University, were retrospectively analyzed.
Results:
Of the 44 LCH patients, 32 (72.7%) were children, 12 (27.3%) were adults. Of the 32 children patients, 15 (46.9%) were less than 2 years old. The involved organs were bone (20/44, 45.5%), lymph nodes (20/44, 45.5%), skin (16/44, 36.4%), liver and spleen (16/44, 36.4%), lung (14/44, 31.8%), bone marrow (6/44, 13.6%), ear (4/44, 9.1%), eyes (2/44, 4.5%), pituitary gland (2/44, 4.5%), and thymus gland (1/44, 2.3%). Of the 44 patients, 10 had 1 organ involved, 11 had 2 organs involved, 9 had 3 organs involved, 7 had 4 organs involved, 7 had no less than 5 organs involved; 22 children patients and 4 adult patients had multiple organs (liver, lung, bone marrow, and so on) involved; 26 (59.1%) were misdiagnosed as dermatosis (6 cases), hemopathy (7 cases), bone or bone marrow diseases (7 cases), lung tuberculosis (2 cases), diabetes insipidus (2 cases), ear or eye disease (2 cases). Six children patients had bronchopneumonia, and 4 of them had septicemia. Six patients died with 5 cases below the age of 2.
Conclusions:
LCH is easy to be misdiagnosed. Children below 2 years old tend to complicate with multiple organ failure (MOF) and bronchopneumonia which have high death rate. Pathology examination is helpful for early diagnosis, treatment, and prevention of severe complications.
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