It is regarded as a clonal accumulation and proliferation of abnormal bone marrow derived langerhans cells. Jun 21, 2006 langerhans cell sarcoma lcs is a neoplastic proliferation of langerhans cells that have overtly malignant cytologic features 1,2. Shortness of breath, cough, and chest pains are common symptoms. Involvement manifestation possible other condition skin vesicles and bullae most common in early infancy erythema toxicum herpes simplex chickenpox infection dermatitis most frequently scalp, diaper or axilla, may occur up to late infancy seborrheic dermatitis. There may be regression of the disease and improvement in symptoms with smoking cessation but with. 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. A bstract background pulmonary langerhanscell histiocytosis is an uncommon interstitial lung disease in adults. Bone pain, increased thirst, increased urine, and rash may be seen. Pulmonary langerhans cell histiocytosis radiology case. Other symptoms include constitutional symptoms fatigue and weight losspleuritic chest pain, or spontaneous pneumothorax pulmoonar. It has some characteristics of cancer but, unlike almost every other cancer, it may spontaneously resolve in some patients while being lifethreatening in others.
Symptoms are dyspnea, cough, fatigue, and pleuritic chest pain. The lesions of langerhans cell histiocytosis have a wide spectrum of histologic changes, but a constant feature is a sinusoidal proliferation of langerhans cells in the appropriate cellular background fig. Langerhans cell histiocytosis lch treatment may include observation alone, surgery, radiation therapy, or oral, topical, and intravenous medication. Langerhans cell histiocytosis causes, symptoms, diagnosis. Langerhans cell histiocytosis an overview sciencedirect. Pulmonary langerhans cell histiocytosis plch is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. Langerhans cell histiocytosis is a rare systemic disease. Editorial from the new england journal of medicine langerhans cell histiocytosis. Langerhans cell sarcoma arising from langerhans cell. Its clinical course is typically characterized by stabilization or regression of bilateral micronodular infiltrates seen on chest radiographs. Langerhans cell sarcoma lcs is a neoplastic proliferation of langerhans cells that have overtly malignant cytologic features 1,2. 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.
Plch can be one manifestation of langerhans cell histiocytosis, a multisystem disease characterized by infiltration of cells called histiocytes. Erdheimchester disease is a rare form of histiocytosis affecting mainly adults that involves multiple parts of the body. Langerhans cell histiocytosis with spinal, pulmonary and pituitary involvement. Pulmonary langerhans cell histiocytosis plch is an idiopathic interstitial lung disease with langerhans cell infiltration in the lung. Langerhans cell histiocytosis hematology and oncology. 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. Etiology is unknown, but cigarette smoking plays a primary role.
The symptoms experienced by patients depend on the organs that are affected 1, 2. Lch is classified as a cancer and sometimes requires treatment with chemotherapy. Symptoms range from isolated bone lesions to multisystem disease. Langerhans cell histiocytosishealth professional version. Pulmonary langerhans cell histiocytosis is a disease in which monoclonal cd1apositive langerhans cells a type of histiocyte infiltrate the bronchioles and alveolar interstitium, accompanied by lymphocytes, plasma cells, neutrophils, and eosinophils. Adult langerhans cell histiocytosis with pulmonary and colorectoanal involvement. Aug 28, langerhans cell histiocytosis lch is a rare histiocytic disorder most in erdheimchester disease but not in other non langerhans cell. Langerhans cells, which help regulate the immune system, are normally found throughout the body, especially in the skin, lymph nodes, spleen, lungs, liver, and bone marrow. Langerhans cell histiocytosis lch is a rare histiocytic disorder of unknown etiopathogenesis. There is a spectrum of these disorders ranging from benign cell proliferations to obviously malignant tumors.
It chiefly affects young adults, primarily occurring in. Pulmonary langerhans cell histiocytosis pulmonary disorders. Histiocytosis describes a diverse group of proliferative disorders involving dendritic cells or macrophages. These dendritic cells along with lymphocytes, eosinophils and normal histiocytes form infiltrates typical for the disease which may be found in various. Some forms of the disorder are genetic, which means they are inherited. Analyses of plch tissues have identified activating mutations of specific mitogenactivated protein kinases. What are the symptoms of pulmonary langerhans cell. In adults, plch is frequently isolated and affects young smokers of both sexes. 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. Lch is part of a group of syndromes called histiocytoses, which are characterized by an abnormal proliferation of histiocytes an. Clinical outcomes of pulmonary langerhanscell histiocytosis. Adult pulmonary langerhans cell histiocytosis plch is a rare disorder of unknown etiology that occurs predominantly in young smokers, with a peak incidence at 2040 years of age. Lichtenstein in 1952 to embrace three disorders characterized by accumulation of histiocytes in different tissues. Langerhans cell histiocytosis lch is a very rare form of disorder that involves clonal proliferation of the langerhans cells, the abnormal cells that are derived from the bone marrow which are capable of moving from the skin to the lymph nodes.
Diagnosis is based on history and imaging tests and sometimes on bronchoalveolar. Nov 08, 2018 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. Dec 16, 2012 langerhans cell histiocytosis lch is a very rare form of disorder that involves clonal proliferation of the langerhans cells, the abnormal cells that are derived from the bone marrow which are capable of moving from the skin to the lymph nodes. 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 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. Pathologic findings were consistent with pulmonary langerhans cell histiocytosis lch.
Adult pulmonary langerhans cell histiocytosis thorax. In 1868, paul langerhans discovered the epidermal dendritic ce. To date, no largescale studies have been done on how often lch occurs in. While the overwhelming majority of patients are smokers, mechanisms. The medical condition known as langerhans cell histiocytosis is a rare disorder that may cause damage to other organs of the body. Pulmonary langerhans cell histiocytosis radiographics. Langerhans cell histiocytosis is a rare disorder that can affect people of all ages. Current understanding and management of pulmonary langerhans. Pulmonary langerhans cell histiocytosis has variable appearance depending on the stage of disease, ranging from small peribronchiolar nodular opacities to multiple irregularlyshaped cysts. Langerhans cell histiocytosis lch is a rare disease that may affect any age group. Adult langerhans cell histiocytosis with pulmonary and. Get detailed treatment information for lch in this summary for clinicians.
Pulmonary langerhans cell histiocytosis orphanet journal. Pulmonary langerhans cell histiocytosis plch, also called eosinophilic granuloma of the lung, pulmonary langerhans cell granulomatosis, or pulmonary histiocytosis x, is an interstitial lung disease that occurs most often in young adults. Continuing navigation will be considered as acceptance of this use. Pulmonary langerhans cell histiocytosis plch is an uncommon but important cause of interstitial lung disease, and it occurs predominantly in adult cigarette smokers.
It can cause distinct clinical syndromes that have been historically described as eosinophilic granuloma, handschullerchristian disease, and letterersiwe disease. Histiocytosis uk is a noncommercial partner of the national institute for health research appropriate research studies funded through histio uks eligible funding streams research project grants are now automatically eligible for nihr clinical research network crn support and therefore entitled to access nhs support via the nihr clinical research network. Treatment depends on the site and extent of disease. Langerhans cell histiocytosis genetics home reference nih. Langerhans cell histiocytosis womens and childrens. While langerhans cells are found in normal, healthy people, there is an overproduction and buildup of these cells which can.
Its clinical presentation is variable and ranges from isolated skin or bone disease to a lifethreatening multisystem condition. Histiocyte cells are a form of white blood cells that help the immune system destroy foreign materials and fight infection. Pulmonary langerhans cell histiocytosis plch is an orphan disease in respiratory medicine, which most affects adult smokers. Adult pulmonary langerhans cell histiocytosis lch is a rare disorder of unknown aetiology that occurs predominantly in young smokers, with an incidence peak at 2040 years of age 1, 2. The langerhans cell is an antigenprocessing cell found in the skin, mucous membranes, including the surface of the eye, lymph nodes, and spleen. Plch belongs to the spectrum of langerhans cell histiocytosis lch, diseases characterized by uncontrolled proliferation and infiltration of various organs by langerhans cells. These lungrelated symptoms may be accompanied by fever, fatigue, and weight loss. Langerhans cell histiocytosis lch is a dendritic cell antigenpresenting cell disorder. Symptoms of pulmonary langerhans cell histiocytosis plch may vary from person to person.
Pulmonary langerhans cell histiocytosis forms part of a spectrum of diseases characterized by monoclonal proliferation and infiltration of organs by langerhans cells table 1. The french langerhans cell histiocytosis study group 1996 a multicentre retrospective survey of langerhans cell histiocytosis. Langerhans cell histiocytosis with spinal, pulmonary and. It is caused by a disorder of myeloid dendritic cells.
Langerhans refers to the doctor who first described normal langerhans cells. Langerhans cell histiocytosis also called histiocytosis x is a rare disorder that primarily affects children. The discoveries that these cells are not confined to skin and that they make up a sizable portion of. Find evidencebased information on langerhans cell histiocytosis treatment. Objectives to describe the morphologic characteristics of skin lesions, extent of extracutaneous disease, and outcomes in patients with neonatal presentation of langerhans cell histiocytosis lch, and to examine clinical predictors of disease prognosis. 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. Pulmonary langerhans cell histiocytosis mayo clinic. While the overwhelming majority of patients are smokers, mechanisms by. Eosinophilic granuloma, also known as pulmonary histiocytosis x phx or pulmonary langerhans cell histiocytosis x plch, is an uncommon interstitial lung disease that is epidemiologically related to tobacco smoking. Langerhans cell histiocytosis clinical presentation. Langerhans cell histiocytosis information from the histiocytosis association. Langerhans cell histiocytosis lch is a rare disease involving clonal proliferation of dendritic cells and macrophages, both of which belong to mononuclear phagocytic system, and affects multiple organs. Its its clinical presentation is variable and ranges from isolated skin or bone disease to a lifethreatening multisystem.
Langerhans cell histiocytosis is a disorder in which excess immune system cells called langerhans cells build up in the body. 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. The purpose of this article was to discuss the clinical features, especially the radiologic features of plch patients during their hospitalization through a. Although pulmonary involvement in lch may be part of a multisystem disease 35, pulmonary lch plch occurs frequently as a singlesystem disease in adults 1, 2. The langerhans cell is a dendritic cell dc of the epidermis that was first described by a medical student, paul langerhans, who thought it was part of the nervous system langerhans, 1868. Pulmonary langerhans cell histiocytosis plch may be seen as part of widespread involvement in patients with disseminated lch or more frequently as a distinct entity in young adult smokers.
Acute progressive histiocytosis x a specific type of histiocytosis x. Objectives to describe the morphologic characteristics of skin lesions, extent of extracutaneous disease, and outcomes in patients with neonatal presentation of langerhans cell histiocytosis lch, and to examine clinical predictors of disease prognosis design retrospective validation cohort study. Histiocytic diseases, like langerhans cell histiocytosis lch and myeloidderived dendritic cell disorder, in children and adults are caused by an abnormal accumulation of cells of the mononuclear phagocytic system. Differential diagnosis for manifestations of langerhans cell histiocytosis. Ryan1, vijayakumar ganesh1, afroditi karathanasi1, michele moschetta2, matin sheriff1, john b. Pulmonary langerhans cell histiocytosis plch is proliferation of monoclonal langerhans cells in lung interstitium and airspaces. Pulmonary langerhans cell histiocytosis lch is a form of langerhans cell disease that primarily affects smokers in the third to fifth decade. The name of the disease is derived from the type of body cell involved, the histiocyte, osis meaning increased numbers.
Pulmonary langerhans cell histiocytosis orphanet journal of. What about acth deficiency without diabetes insipidus. Axial lung window there are multiple centrilobular nodules present in both lungs. Langerhans cell histiocytosis presenting in the neonatal. Extrathoracic investigation in adult patients with. 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. Lch can occur at any age but is more frequent in the pediatric population. Within this spectrum exist several proliferative conditions involving the langerhans cell, which, historically, have been variably classified, causing nosologic confusion. Plch x has nonspesific symptoms, and most patients have. Langerhans cell histiocytosis is a rare disease, which can affect children of any age.
In langerhans cell histiocytosis, excess immature langerhans cells usually form tumors. Pulmonary langerhans cell histiocytosis plch is a diffuse lung disease that usually affects young adult smokers. Langerhans cell histiocytosis lch is a term for a variety of diseases characterised by the proliferation and infiltration of langerhans cells lcs into various organs 3, 4. Langerhans cell histiocytosis lch lch is an unusual condition. How i manage pulmonary langerhans cell histiocytosis. This disorder is characterized by an abnormal increase in certain immune cells called histiocyte cells. A histiocyte is a normal white blood cell, which is part of the.
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