Clinical profile, disease-distribution and outcome of Langerhans cell histiocytosis (LCH) is presented in this retrospective analysis. There were 69 children with LCH from January 1986 to December 2004. Diagnosis was presumptive in the majority. The age ranged from 2 months to 12 years. Multisystem disease was documented in 48 (69.6%) children. Evidence of hepatic dysfunction was detected in 25 (36.2%). An elevated serum alkaline phosphatase was a prominent observation in patients with hepatic involvement. Children with localized disease received oral steroids, while cases with disseminated/multi-system disease were treated with prednisolone and, vinblastine or etoposide. 20 (54%) children with disseminated disease and organ dysfunction died. A favorable outcome was documented in all but one case with localized disease. Portal hypertension developed in 3 cases, all of whom had a fatal outcome. Twelve (17.4%) patients had diabetes insipidus. Disseminated disease with organ dysfunction was observed to be a predictor of fatal outcome.
Download full-text PDF |
Source |
|---|
Publication Analysis
Top Keywords
Similar Publications
High resolution chest computed tomography responses for a cohort of adult with pulmonary Langerhans cell histiocytosis.
Ann Hematol
January 2025
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, People's Republic of China.
Langerhans cell histiocytosis (LCH) is a heterogeneous histiocytosis with various pulmonary manifestations and imaging. We aim to evaluate the pulmonary response of LCH by high resolution chest computed tomography (HRCT) through continuous follow-up.We conducted a retrospective analysis of 73 adult LCH patients with pulmonary involvement.
View Article and Find Full Text PDFCirculating Tumor DNA Combining with Imaging Analysis for Lesion Detection of Langerhans Cell Histiocytosis in Children.
Children (Basel)
November 2024
Department of Pediatrics, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, China.
Background: The detection of mutations from circulating tumor DNA (ctDNA) represents a promising enrichment technique. In this retrospective study, the significance of ctDNA and imaging in Langerhans cell histiocytosis (LCH) monitoring was first examined, and the broader role of ctDNA in monitoring LCH was additionally explored.
Methods: First, data visualization and survival analysis models were used to generalize the concordance between cfBRAF molecular response and radiographic response on clinical outcomes.
Histiocytosis are caused by pathogenic myeloid cells, and can be classified as Langerhans cell histiocytosis (LCH) and non-LCH. Erdheim-Chester disease (ECD) is a non-LCH, characterized by multi-organ involvement, typical imaging findings, and confirmatory histological studies. A case with multi-organ involvement and histological confirmation is presented.
View Article and Find Full Text PDFAdult-onset Langerhans cell histiocytosis with multisystem involvement: A rare case report.
Radiol Case Rep
March 2025
Radiology Department, Hamad Medical Corporation, Doha, Qatar.
Article Synopsis
- Langerhans cell histiocytosis (LCH) is a rare disorder marked by the abnormal buildup of dendritic histiocytes in tissues, often presenting as single- or multi-system diseases, particularly affecting adults.
- In a reported case, a 46-year-old woman experienced back pain, motor and sensory deficits, and was diagnosed with a compression fracture in the spine, alongside signs of central nervous system and salivary gland involvement.
- The patient underwent surgical intervention that included decompression and a biopsy, confirming the presence of LCH, highlighting the complexity and rarity of adult-onset LCH with extensive system involvement.
Erdheim-Chester disease: Comprehensive insights from genetic mutations to clinical manifestations and therapeutic advances.
Dis Mon
January 2025
Department of Pharmaceutical Sciences, Babasaheb Bhimrao Ambedkar University, Vidya Vihar, Raebareli Road, Lucknow-226025, (U.P.), India. Electronic address:
Erdheim-Chester disease (ECD) is an extremely rare non-Langerhans cell disorder that is believed to include both inflammatory and neoplastic characteristics. It is caused due to genetic mutations in proto-oncogenes like BRAF and MEK, while immunological pathways have an essential role in the onset and progression of the disease. Despite its rarity, ECD poses significant diagnostic and therapeutic challenges due to its heterogeneous clinical presentation and limited understanding of its underlying pathophysiology.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!