A comprehensive diagnostic approach, including immunohistochemistry, is crucial for confirming pulmonary Langerhans cell histiocytosis in adults. Individualized treatment with dynamically adjusted chemotherapy based on therapeutic response leads to significant absorption of lesions and symptom alleviation. Regular follow-up and timely treatment adjustments according to the patient's condition are essential in managing this rare disease.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11331026 | PMC |
| http://dx.doi.org/10.1002/ccr3.9342 | DOI Listing |
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 PDFErdheim-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 PDFA case of adult-onset X-linked lymphoproliferative disease mimicking pulmonary infection.
BMC Infect Dis
December 2024
National Center for Respiratory Medicine, China-Japan Friendship Hospital, Beijing, 100029, China.
X-linked lymphoproliferative disease (XLP) is a rare primary immunodeficiency with susceptibility and vulnerability to Epstein-Barr virus (EBV) infection. Most patients were diagnosed in early childhood and do not survive into adulthood. Here we reported an adult-onset XLP patient presenting with fever, dyspnea, and pulmonary nodules, mimicking respiratory infection at disease onset.
View Article and Find Full Text PDFA case of Erdheim-Chester disease-a mimicker of IgG4-related disease and large vessel vasculitis.
Mod Rheumatol Case Rep
December 2024
The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Japan, Kitakyushu, Japan.
The patient was a 57-year-old man who developed bilateral thigh pain and chest tightness one year ago. Chest CT scan showed reticular shadows, thickened interlobular septa in both lung fields, and pericardial effusion. Three months ago, his symptoms worsened.
View Article and Find Full Text PDFPosterior Instrumentation without Curettage Promotes Rapid Restoration of Adult Spinal Langerhans Cell Histiocytosis.
Spine Surg Relat Res
November 2024
Department of Orthopaedics, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
Introduction: Adult spinal Langerhans cell histiocytosis (LCH) presents a treatment challenge due to ongoing controversies. Traditional approaches such as curettage with bone grafting and internal fixation are preferred for severe cases involving mechanical instability, neurological deficits, or deformity. This study aimed to explore the efficacy of a customized approach involving simple posterior instrumentation without curettage or bone grafting in treating adult spinal LCH.
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!