Langerhans cell histiocytosis lch is a rare cancer involving clonal proliferation of langerhans cells, abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes. Primary pulmonary langerhans cell histiocytosis associated with smoking in an adolescent boy. It is caused by a disorder of myeloid dendritic cells. In 1868, paul langerhans discovered the epidermal dendritic ce. Clinical spectrum of langerhans cell histiocytosis.
Pulmonary lch seems to be a distinct entity, as it occurs. Pdf pulmonary langerhans cell histiocytosis plch is an unusual cystic lung disease that is also characterized by. Age distribution of patients with langerhans cell histiocytosis lch. Clinical research of pulmonary langerhans cell histiocytosis in. Lch is part of a group of syndromes called histiocytoses, which are characterized by an abnormal proliferation of histiocytes an. Severe pulmonary arterial hypertension pah complicating plch is rare who classification type 5 and optimal management strategies of these patients remain unknown. Histiocyte is now considered an overarching term to describe cells of dendritic cell dc or macrophage lineage. Etiology is unknown, but cigarette smoking plays a primary role. Pulmonary langerhans cell histiocytosis plch is a smoking. Treatment depends on the site and extent of disease.
Histiocyte cells are a form of white blood cells that help the immune system destroy foreign materials and fight infection. Pulmonary langerhans cell histiocytosis springerlink. Primary pulmonary langerhans cell histiocytosis associated. In adults, pulmonary involvement with langerhans cell histiocytosis usually occurs as a singlesystem disease and. Pulmonary langerhans cell histiocytosis pulmonary disorders. Initial and final ct scans of 21 patients with lch and ct evidence of pulmonary disease were compared retrospectively. To document the evolution of pulmonary lesions of langerhans cell histiocytosis lch with sequential computed tomography ct.
Pulmonary langerhans cell histiocytosis plch is an unusual cystic lung disease that is also characterized by extrapulmonary manifestations. But in histiocytosis histeeohsytoesis, the cells grow so quickly that they build up. Langerhans cell histiocytosis msd manual consumer version. They are found in the skin, lymph nodes, spleen, bone marrow and lungs. Pulmonary langerhans cell histiocytosis plch is a rare lung disease caused by the accumulation of inflammatory cells in small airways of the lung, leading to nodular granulomatous changes of the lung parenchyma, which over time, progress to reticular and cystic changes. The purpose of this article was to discuss the clinical features, especially the radiologic features of plch patients during their hospitalization through a retrospective analysis on clinical data. Langerhans cell histiocytosis lch is a rare disorder characterized by dysregulated growth, activity, and trafficking of langerhans cells. Plch belongs to the spectrum of langerhans cell histiocytosis lch, diseases characterized by uncontrolled proliferation and infil. Request pdf pulmonary langerhans cell histiocytosis langerhans cell histiocytosis is a rare idiopathic disorder, characterized by the infiltration of 1 or more organs by large mononuclear cells. Pulmonary langerhans cell histiocytosis plch is an uncommon but important cause of interstitial lung disease, and it occurs predominantly in adult cigarette smokers 1. While the overwhelming majority of patients are smokers, mechanisms. Symptoms are dyspnea, cough, fatigue, and pleuritic chest pain. The relation of langerhans cell histiocytosis to acute.
Complete pathological resolution of pulmonary langerhans cell. So far, the patients have not presented any organ involvement by lch. Langerhans cell histiocytosis information from the histiocytosis association. Pulmonary langerhans cell histiocytosis plch is an interstitial primary. Determinants of survival in pulmonary langerhans cell granulomatosis histiocytosis x. Pulmonary langerhans cell histiocytosis is a chronic lung disease that results in the formation of multiple spherical or irregularly shaped, thinwalled, airfilled spaces within the lung parenchyma. To date, no largescale studies have been done on how often lch occurs in. Recent multicentre studies have led to the more standardised management of patients. Adult pulmonary langerhans cell histiocytosis thorax.
Pulmonary langerhans cell histiocytosis plch is an orphan disease in respiratory medicine, which most affects adult smokers. Langerhans cell histiocytosis lch in adults is a rare disorder that occurs when the body produces too many langerhans cells histiocytes, which are a type of white blood cell that helps fight infection. Diagnosis is based on history and imaging tests and sometimes on bronchoalveolar. Langerhans cell histiocytosis seattle childrens hospital. The cellular spectrum covered by the term histiocyte has evolved over time. Chest xrays show nodules, small thickwalled lung cysts, and other changes that are typical of pulmonary langerhans cell histiocytosis.
Medical management of langerhans cell histiocytosis from. Pulmonary langerhans cell histiocytosis mayo clinic. Pulmonary langerhans cell histiocytosis plch is an uncommon disorder of adult smokers associated with a significant morbidity. Alpha1 and pulmonary langerhans cell histiocytosis.
It is a monthly journal that publishes a total of 12 issues, which contain these types of articles to different extents. Langerhans cell histiocytosis nanette grana, md background. Continuing navigation will be considered as acceptance of this use. Computed tomography ct may show these changes in enough detail to establish the diagnosis. Langerhans cell histiocytosis also called histiocytosis x is a rare disorder that primarily affects children.
In adults, plch is frequently isolated and affects young smokers of both sexes. Aug 28, langerhans cell histiocytosis lch is a rare histiocytic disorder most in erdheimchester disease but not in other non langerhans cell. Pulmonary langerhans cell granulomatosis histiocytosis x. Langerhans cell histiocytosis lch is a rare histiocytic disorder most commonly characterized by single or multiple osteolytic bone lesions or skin rashes demonstrating infiltration with histiocytes with beanshaped nuclei on biopsy with or without histiocytic infiltration of extraskeletal tissues most notably the skin, lungs, lymph nodes, liver, spleen, bone marrow, brain. Arising from the aberrant accumulation of langerhans and other immune cells, plch tends to cause a relatively isolated pulmonary involvement as compared to other forms of langerhans cell lc and histiocytic disorders. Other symptoms include constitutional symptoms fatigue and weight losspleuritic chest pain, or spontaneous pneumothorax pulmoonar. Langerhans cell histiocytosis lch is a group of idiopathic disorders characterized by the presence of cells with characteristics similar to bone marrowderived langerhans cells juxtaposed against a backdrop of hematopoietic cells, including tcells, macrophages, and eosinophils. Pulmonary langerhans cell histiocytosis plch is a rare sporadic cystic lung disease of. Pulmonary langerhans cell histiocytosis plch is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette. Vassallo r, ryu jh, colby tv, hartman t, limper ah. Pulmonary lch in adults is a rare disease and no precise epidemiological data are available concerning its prevalence.
This disorder is characterized by an abnormal increase in certain immune cells called histiocyte cells. Pulmonary langerhans cell histiocytosis definition of. The incidence is one to two cases per million adults. Pulmonary langerhans cell histiocytosis request pdf. While langerhans cells are found in normal, healthy people, there is an overproduction and buildup of these cells which can lead to organ. In langerhans cell histiocytosis, certain white blood cells langerhans cells grow out of control. Review open access pulmonary langerhans cell histiocytosis harpreet s suri1, eunhee s yi2, gregorz s nowakowski3 and robert vassallo1,4 abstract pulmonary langerhans cell histiocytosis plch is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. Table 3 the dominant clinical feature at the time of each patients presentation. Pathologic findings were consistent with pulmonary langerhans cell histiocytosis lch. Pulmonary lch plch is a rare disorder occurring in adults that is associated with cigarette smoking. Adult pulmonary langerhans cell histiocytosis is a rare disorder of unknown aetiology that occurs predominantly in young smokers, with an incidence peak at 2040 yrs of age. Pulmonary langerhans cell histiocytosis is a rare granulomatous disease affecting both sexes, with greater incidence in the second and third decades of life.
Successful treatment of adult multisystemic langerhans. Langerhans cells are white blood cells in the immune system that normally play an important role in protecting the body against viruses, bacteria and other invaders. Langerhanscell histiocytosis a spectrum of diseases formerly known as histiocytosis x and now called langerhanscell histiocytosis is characterized by erk pathway activation including braf v600e. Subsequently, he has been free from pneumothorax during the 2 years of follow up.
Pulmonary langerhans cell histiocytosis plch, previously called eosinophilic granuloma of the lung, pulmonary langerhans cell granulomatosis, and pulmonary histiocytosis x, is an uncommon cystic interstitial lung disease that primarily affects young adults. Langerhans cell histiocytosis lch treatment may include observation alone, surgery, radiation therapy, or oral, topical, and intravenous medication. How i manage pulmonary langerhans cell histiocytosis european. Discussion dendritic cells are components of the mammalian immune system that are chiefly located in tissues that contact the external environment, such as the skin, lungs, and gastrointestinal tract. Mutations in signalling molecules that increase cell survival and proliferation via the mitogen activating protein kinase mapk pathway have been identified in systemic and.
Get detailed treatment information for lch in this summary for clinicians. Pulmonary langerhans cell histiocytosis is an uncommon interstitial lung disease that results from the accumulation of cells known as langerhans cells in the lung. Targeted therapy with braf inhibitors has been used in select patients with lch, and the results have been encouraging. Langerhans cell histiocytosis lch is a rare histiocytic disorder of unknown etiopathogenesis. Langerhans cell histiocytosis clinical presentation. Symptoms range from isolated bone lesions to multisystem disease.
The term pulmonary langerhans cell histiocytosis is used to refer to disease in adults that affects the lung, either in isolation or in addition to other organ systems. Pulmonary langerhans cell histiocytosis plch is proliferation of monoclonal langerhans cells in lung interstitium and airspaces. Review open access pulmonary langerhans cell histiocytosis. There may be regression of the disease and improvement in symptoms with smoking cessation but with. Adult pulmonary langerhans cell histiocytosis european. Pulmonary langerhans cell histiocytosis plch is a rare disease with insidious onset and nonspecifi. Langerhans cell histiocytosis is a rare disease that includes a. Pulmonary langerhans cell histiocytosis radiology case. Langerhans cell histiocytosis lch is a rare histiocytic disorder characterized by infiltration by histiocytes, which may infiltrate nearly every organ, including lungs and lymph nodes. All patients n 5 314 are compared with patients with multisystem lch n 5 96, patients with isolated osseous lch n 5 114, and patients with isolated pulmonary lch n 5 87. In langerhans cell histiocytosis formerly known as histiocytosis x, langerhans cells multiply abnormally. How i manage pulmonary langerhans cell histiocytosis gwenael lorillon1 and abdellatif tazi1,2 affiliations. Pulmonary langerhans cell histiocytosis orphanet journal.
Frontal the plain frontal chest xray shows mild hyperinflation and a coarse pattern in the lung fields consisting of reticular interstitial markings and peripheral ring shadows. Pulmonary langerhans cell histiocytosis with inguinal. Pulmonary langerhans cell histiocytosis radiographics. In adults, pulmonary involvement with langerhans cell histiocytosis usually occurs as a singlesystem disease and is characterised by focal langerhans cell granulomas infiltrating and destroying distal bronchioles. 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. Pulmonary langerhans cell histiocytosis plch is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. Pulmonary langerhans cell histiocytosis chest foundation. In a healthy person, langerhans langerhanz cells help fight infection.
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