The diagnosis of pulmonary Langerhans cell histiocytosis might be refined by demonstrating reliability of a new cell marker, i.e., Langerin (CD207), used on bronchoalveolar lavage fluid. For this purpose, we collected material from patients with this disease and also with sarcoidosis and idiopathic pulmonary fibrosis as controls. In addition to the immunocytochemical detection of Langerin, we examined the expression profiles of CD1a and the macrophage tandem-repeat mannose receptor (CD206). To test accessibility of Langerin, a C-type lectin, for mannosides, we employed reverse lectin histochemistry using mannose-containing neoglycoproteins. The analysis revealed a significantly increased percentage of CD1a- and Langerin-positive cells in pulmonary Langerhans cell histiocytosis in comparison with both other studied diseases. No expression of the 175-kDa mannose-binding lectin (CD206) in Langerhans cells was observed. Evidently, binding sites on the cells were not accessible for the mannose-containing neoglycoligand. These results provide evidence for the usefulness of Langerin-directed immuno- and glycohistochemical monitoring of bronchoalveolar lavage fluid in the diagnosis of pulmonary Langerhans cell histiocytosis.
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http://dx.doi.org/10.1007/s00428-003-0952-6 | DOI Listing |
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 PDFMod 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 PDFSpine 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 PDFArch Clin Cases
December 2024
Department of Medicine, Queensland Health, Mackay, Queensland, Australia.
Pulmonary Langerhans cell histiocytosis (PLCH) in adults is an uncommon disorder that occurs almost exclusively in smokers. PLCH has no known gender predilection, and the current consensus of its true aetiology is unknown. Lungs may the sole organ involved, however other organs in the body may be involved as well.
View Article and Find Full Text PDFRev Paul Pediatr
December 2024
Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto, Ribeirão Preto, SP, Brazil.
Objective: The objective of this study was to report two cases of successive multiple spontaneous bilateral pneumothorax in children with massive lung involvement due to Langerhans cell histiocytosis (LCH), emphasizing the possibility of this differential diagnosis for the general pediatrician. Additionally, published cases describing pediatric patients with pulmonary manifestations of LCH were reviewed in the literature.
Case Description: Case #1: A 3-year-old male patient with a sudden episode of spontaneous right-sided pneumothorax, surgically drained.
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