The Experts below are selected from a list of 318 Experts worldwide ranked by ideXlab platform
A Tazi - One of the best experts on this subject based on the ideXlab platform.
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the natural history of adult pulmonary Langerhans Cell histiocytosis a prospective multicentre study
Orphanet Journal of Rare Diseases, 2015Co-Authors: A Tazi, Constance De Margerie, Jean Marc Naccache, S Dominique, Stephane Jouneau, Gwenael Lorillon, Emmanuelle Bugnet, R Chiron, B Wallaert, Dominique ValeyreAbstract:Background The natural history of pulmonary Langerhans Cell histiocytosis (PLCH) has been unclear due to the absence of prospective studies. The rate of patients who experience an early progression of their disease is unknown. Additionally, conflicting effects of smoking cessation on the outcome of PLCH have been reported.
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adult pulmonary Langerhans Cell histiocytosis
European Respiratory Journal, 2006Co-Authors: A TaziAbstract:Adult pulmonary Langerhans’ Cell histiocytosis is a rare disorder of unknown aetiology that occurs predominantly in young smokers, with an incidence peak at 20–40 yrs of age. In adults, pulmonary involvement with Langerhans’ Cell histiocytosis usually occurs as a single-system disease and is characterised by focal Langerhans’ Cell granulomas infiltrating and destroying distal bronchioles. High-resolution computed tomography (HRCT) of the chest is essential to the diagnosis, typically showing a combination of nodules, cavitated nodules, and thick- and thin-walled cysts. A high macrophage count in bronchoalveolar lavage (BAL) fluid is a common but nonspecific finding that merely reflects exposure to tobacco smoke. BAL is useful for eliminating infections and the other infiltrating lung disorders that can be seen in young adults. Langerhans’ Cells can be identified in BAL fluid, but, in contrast to what was initially hoped, this test shows a very low sensitivity and is rarely useful in the diagnosis of the disease. The definite diagnosis of pulmonary Langerhans’ Cell histiocytosis requires identification of Langerhans’ Cell granulomas, which is usually achieved by surgical lung biopsy at a site selected by chest HRCT. In practice, however, lung biopsy is performed on a case-by-case basis. No effective treatment is available to date, and improved understanding of the mechanisms involved in the pathogenesis of pulmonary Langerhans’ Cell histiocytosis is urgently needed, and should help in the development of specific therapeutic strategies for patients with this orphan disease.
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Pulmonary Langerhans Cell granulomatosis.
Current Opinion in Pulmonary Medicine, 1995Co-Authors: Paul Soler, A Tazi, Allan J. HanceAbstract:Pulmonary Langerhans Cell granulomatosis (LCG), also called histiocytosis X, is characterized by the presence of destructive granulomas containing large numbers of Langerhans Cells. The lesions are almost exclusively centered on distal bronchioles, and it may be more accurate to consider the disease as a bronchiolitis. Isolated pulmonary LCG is an uncommon disease that usually affects young adult smokers. Multifocal or diffuse involvement can also occur, though more often in infants or children. High-resolution computed tomography has proved to be useful in the diagnosis of the disease by allowing the identification of characteristic cystic lesions associated with nodules. Bronchoalveolar lavage strongly supports the diagnosis in only the occasional patient. The pathogenesis of pulmonary LCG remains unknown, although several arguments suggest that, at least in adults, it may result from an uncontrolled immune response initiated by Langerhans Cells. Granulocyte macrophage colony-stimulating factor could be one of the factors responsible for the local accumulation of Langerhans Cells in early lesions. Recently, evidence that lesional Langerhans Cells are of clonal origin has been reported in patients who have various forms of the disease, but this finding has not yet been shown for pulmonary LCG. Further studies are needed to determine whether the pathogenetic mechanisms are different in patients who have localized or diffuse forms of the disease.
Robert Vassallo - One of the best experts on this subject based on the ideXlab platform.
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pulmonary Langerhans Cell histiocytosis
Orphanet Journal of Rare Diseases, 2012Co-Authors: Harpreet S Suri, Eunhee S Yi, Gregorz S Nowakowski, Robert VassalloAbstract:Pulmonary Langerhans Cell Histiocytosis (PLCH) is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. The pathologic hallmark of PLCH is the accumulation of Langerhans and other inflammatory Cells in small airways, resulting in the formation of nodular inflammatory lesions. While the overwhelming majority of patients are smokers, mechanisms by which smoking induces this disease are not known, but likely involve a combination of events resulting in enhanced recruitment and activation of Langerhans Cells in small airways. Bronchiolar inflammation may be accompanied by variable lung interstitial and vascular involvement. While Cellular inflammation is prominent in early disease, more advanced stages are characterized by cystic lung destruction, cicatricial scarring of airways, and pulmonary vascular remodeling. Pulmonary function is frequently abnormal at presentation. Imaging of the chest with high resolution chest CT scanning may show characteristic nodular and cystic abnormalities. Lung biopsy is necessary for a definitive diagnosis, although may not be required in instances were imaging findings are highly characteristic. There is no general consensus regarding the role of immunosuppressive therapy in smokers with PLCH. All smokers must be counseled on the importance of smoking cessation, which may result in regression of disease and obviate the need for systemic immunosuppressive therapy. The prognosis for most patients is relatively good, particularly if longitudinal lung function testing shows stability. Complications like pneumothoraces and secondary pulmonary hypertension may shorten life expectancy. Patients with progressive disease may require lung transplantation.
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clinical outcomes of pulmonary Langerhans Cell histiocytosis in adults
The New England Journal of Medicine, 2002Co-Authors: Robert Vassallo, Darrell R Schroeder, Paul A Decker, Andrew H LimperAbstract:Background Pulmonary Langerhans'-Cell histiocytosis is an uncommon interstitial lung disease in adults. It has an unpredictable course and may be associated with an increased susceptibility to the development of malignant neoplasms. Methods We reviewed the records of 102 adults with histopathologically confirmed pulmonary Langerhans'-Cell histiocytosis to ascertain their vital status and whether cancer had been diagnosed. The health status of surviving patients was quantified with the use of the 36-Item Short-Form General Health Survey. Factors potentially associated with survival after the diagnosis of pulmonary Langerhans'-Cell histiocytosis were analyzed with the Cox proportional-hazards model. Results The median follow-up period was 4 years (range, 0 to 23). There were 33 deaths, 15 of which were attributable to respiratory failure. Six hematologic cancers were diagnosed. The overall median survival was 12.5 years, which was significantly shorter than that expected for persons of the same sex and cale...
Lawrence M Weiss - One of the best experts on this subject based on the ideXlab platform.
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immunohistochemical expression of langerin in Langerhans Cell histiocytosis and non Langerhans Cell histiocytic disorders
The American Journal of Surgical Pathology, 2008Co-Authors: Lawrence M WeissAbstract:Langerin is a type II transmembrane C-type lectin associated with the formation of Birbeck granules in Langerhans Cells. Langerin is a highly selective marker for Langerhans Cells and the lesional Cells of Langerhans Cell histiocytosis. Although Langerin protein expression in Langerhans Cell histioc
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pulmonary Langerhans Cell histiocytosis molecular analysis of clonality
The American Journal of Surgical Pathology, 2001Co-Authors: Samuel A Yousem, Thomas V Colby, Yuanyuan Chen, Wen Gang Chen, Lawrence M WeissAbstract:: Pulmonary Langerhans' Cell histiocytosis (LCH) is a form of Langerhans' Cell disease that primarily affects smokers in the third to fifth decade. Extrapulmonary manifestations are rare. Its clinical course is typically characterized by stabilization or regression of bilateral micronodular infiltrates seen on chest radiographs; progression to honeycomb fibrosis is rare. Because the clinical course of pulmonary LCH is distinct from systemic multiorgan LCH, currently thought to be a clonal proliferative disorder, we examined the X-linked polymorphic human androgen receptor assay (HUMARA) locus to assess clonality in female patients with one or more discrete LCH Cell nodules in open lung biopsies. Langerhans' Cells (LCH Cells) were excised from formalin-fixed, paraffin-embedded tissue by microdissection to assure a relatively pure Cellular population, and studies for differential methylation patterns at the HUMARA locus were performed. Twenty-four nodules in 13 patients were evaluated. Seven (29%) were clonal and 17 (71%) were nonclonal. Of six cases with multiple discrete nodules, three (50%) showed a nonclonal LCH Cell population. In one biopsy with five nodules, two nodules were clonal with one allele inactivated, one nodule was clonal with the other allele inactivated, and two nodules were nonclonal. In contrast to systemic LCH, pulmonary LCH appears to be primarily a reactive process in which nonlethal, nonmalignant clonal evolution of LCH Cells may arise in the setting of nonclonal LCH Cell hyperplasia. Cigarette smoking may be the stimulus for pulmonary LCH in contrast to other forms of LCH.
Milen Minkov - One of the best experts on this subject based on the ideXlab platform.
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The coming of age of Langerhans Cell histiocytosis
Nature Immunology, 2020Co-Authors: Carl E. Allen, Matthew Collin, R. Maarten Egeler, Peter C. L. Beverley, Eli L. Diamond, Florent Ginhoux, Christopher Glass, Milen Minkov, Barrett J. Rollins, Astrid HalterenAbstract:Progress in understanding the rare disease Langerhans Cell histiocytosis has stimulated immersive meetings occurring annually over a 30-year period that bring together clinicians, scientists and patients in a unique collaboration.
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therapy prolongation improves outcome in multisystem Langerhans Cell histiocytosis
Blood, 2013Co-Authors: H Gadner, Milen Minkov, Nicole Grois, Ulrike Potschger, Elfriede Thiem, Maurizio Arico, Itziar Astigarraga, Jorge Braier, Jean Donadieu, Janinge HenterAbstract:Langerhans Cell histiocytosis (LCH)-III tested risk-adjusted, intensified, longer treatment of multisystem LCH (MS-LCH), for which optimal therapy has been elusive. Stratified by risk organ involvement (high [RO+] or low [RO–] risk groups), >400 patients were randomized. RO+ patients received 1 to
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multisystem Langerhans Cell histiocytosis in children current treatment and future directions
Pediatric Drugs, 2011Co-Authors: Milen MinkovAbstract:Langerhans Cell histiocytosis (LCH) is a rare (about 3–5 cases per million children aged 0–14 years), non-malignant disease characterized by proliferation and accumulation of clonal dendritic Cells, extreme clinical heterogeneity, and an unpredictable course. Three large-scale, international, prospective therapeutic studies (LCH-I to III) for multisystem LCH (MS-LCH) have been conducted by the Histiocyte Society since 1991. The cumulative lessons from these studies are summarized in this review.
Jeffrey R Galvin - One of the best experts on this subject based on the ideXlab platform.
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from the archives of the afip pulmonary Langerhans Cell histiocytosis
Radiographics, 2004Co-Authors: Gerald F Abbott, Melissa L Rosadodechristenson, Teri J Franks, Aletta Ann Frazier, Jeffrey R GalvinAbstract:: Pulmonary Langerhans Cell histiocytosis (PLCH) is an isolated form of Langerhans Cell histiocytosis that primarily affects cigarette smokers. PLCH is characterized by peribronchiolar proliferation of Langerhans Cell infiltrates that form stellate nodules. The nodular lesions frequently cavitate and form thick- and thin-walled cysts, which are thought to represent enlarged airway lumina. PLCH lesions display temporal microscopic heterogeneity, with progression from dense Cellular nodules to apparently cavitary nodules to increasing degrees of fibrosis that may extend along alveolar walls. In advanced cases, fibrotic scars are surrounded by enlarged, distorted air spaces. Affected patients are typically young adults who often present with cough and dyspnea. The characteristic radiographic features of PLCH are bilateral nodular and reticulonodular areas of opacity that predominantly involve the upper and middle lung zones with relative sparing of the lung bases. High-resolution computed tomography (CT) shows nodules and cysts in the same distribution and allows a confident prospective diagnosis of PLCH in the appropriate clinical setting. In typical cases, a predominantly nodular pattern is seen on CT scans in early phases of the disease, whereas a cystic pattern predominates in later phases. The radiologic abnormalities may regress, resolve completely, become stable, or progress to advanced cystic changes. Treatment consists of smoking cessation, but corticosteroid therapy may be useful in selected patients. Chemotherapeutic agents and lung transplantation may be offered to patients with advanced disease. The prognosis of PLCH is variable with frequent regression, stabilization, or recurrence of disease that does not correlate with cessation or continuation of smoking.
