Sometimes there are mutations changes in lch cells as they form. This disease was previously designated as histiocytosis x, eosinophilic granuloma, letterersiwe disease and many more. Langerhans cell histiocytosis is a disorder in which excess immune system cells called langerhans cells build up in the body. Lch can cause damage to skin, bone and other organs hiv. Langerhans cell histiocytosis lch, or histiocytosis x, is now generally considered to be a nonmalignant condition. Langerhans cell histiocytosis investigations bmj best. 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. Langerhans cell histiocytosis lch has been previously thought of as a rare illness, but is now increasingly diagnosed as a result of the more intensive investigations of patients with cystic pulmonary disease. Langerhans cell histiocytosis lch is a group of rare disorders histologically characterized by the proliferation of langerhans cells.
Langerhans cell histiocytosis lch and lymphangioleiomyomatosis lam medicosis perfectionalis. Langerhans cell histiocytosis is a rare condition that can occur over a wide age range with predominance in males and those under of age of 30. Dendritic cells are a form of histiocyte, or white blood cell. Histiocyte cells are a form of white blood cells that help the immune system destroy foreign materials and fight infection. Langerhans cell histiocytosis lch is the most common dendritic cell disorder and is named because of its similarity to the langerhans cells found in the skin and mucosa. Lch is part of a group of syndromes called histiocytoses, which are characterized by an abnormal proliferation of histiocytes an. Medical management of langerhans cell histiocytosis from. Langerhans cell histiocytosis treatment algorithm bmj. Because treatment concepts are not well defined the german cooperative group on. The uncertainty as to whether langerhans cell histiocytosis is a reactive or a neoplastic disease has resulted in a longstanding debate on this question, and the limited understanding of the. 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. Langerhans cell histiocytosis definition of langerhans. Langerhans cell histiocytosis is a rare condition, slightly more common in boys than girls, affecting around one in 5 million children and fewer adults. Symptoms range from isolated bone lesions to multisystem disease.
It is named after the medical student paul langerhans, the first scientist to. 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. Topical corticosteroids are considered a firstline therapy, particularly for disabling, ulcerative lesions. The most common symptom is pain and swelling over the affected area. 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. Clinical significance langerhans cell histiocytosis. Langerhans cell histiocytosis lch is a rare disease that may affect any age group. Different types of involvement have been described by mri. Langerhans cell histiocytosis genetic and rare diseases. This disorder is characterized by an abnormal increase in certain immune cells called histiocyte cells.
Langerhans cell histiocytosis treatment pdqpatient. Differential diagnosis for manifestations of langerhans cell histiocytosis. Cns changes can have spaceoccupying or degenerative character. Langerhans cell histiocytosis also called histiocytosis x is a rare disorder that primarily affects children.
These dendritic cells along with lymphocytes, eosinophils and normal histiocytes form infiltrates typical for the disease which may be found in various. The medical condition known as langerhans cell histiocytosis is a rare disorder that may cause damage to other organs of the body. The cells build up in the body, sometimes damaging organs or forming tumors. Langerhans cell histiocytoses lch are a rare group of disorders that comprise a large spectrum of diseases initially known as histiocytosis x. The most common sites are the skull, femur, pelvis, and mandible. Plch may be a reactive process with a subset showing clonality, but extrapulmonary forms of langerhans cell histiocytosis are thought to be neoplastic. Langerhans cell histiocytosis is a type of cancer that can damage tissue or cause lesions to form in one or more places in the body. It is regarded as a clonal accumulation and proliferation of abnormal bone marrow derived langerhans cells. In 1973, the name langerhans cell histiocytosis lch was introduced.
Neuropathology of cns disease in langerhans cell histiocytosis. These cells play a role in the bodys immune system. It broadly fits into the category of histiocytoses. Langerhans cell histiocytosis is a clonal proliferation of cells that morphologically and immunophenotypically resemble langerhans cells more common in childhood 1 3 years old and involves nodal and extranodal sites most common site is bone. However, it was later discovered that the abnormal cells in lch are actually derived from myeloid dendritic cells that exhibit the same.
Get detailed treatment information for lch in this summary for clinicians. Etiology is unknown, but cigarette smoking plays a primary role. Langerhans cell histiocytosis lch describes a spectrum of clinical presentations ranging from a single bone lesion or trivial skin rash to an explosive disseminated disease. 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. Histiocytosis x, langerhans cell granulomatosis, multifocal eosinophilic granuloma hematology an autonomous proliferation of a lymphoreticular cell, the langerhans cell, which stains positively with antibodies to atpase, s100 and cd1a. Langerhans cell histiocytosis, abbreviated lch, is a rare disorder of tissue macrophages. Langerhans cell histiocytosis lch represents a rare benign disorder, previously designated as histiocytosis x, type ii histiocytosis or langerhans cell granulomatosis. The histiocytes attack different tissues of the body.
Clinical presentation is heterogeneous and ranges from selfh. It occurs when a persons body makes too many immune cells called histiocytes. However lch cells stain positive to cd14 which is a monocyte marker and shows a different, hematopoietic origin for the disorder. The newer term is preferred as its more descriptive of its cellular background, and removes the ambiguity of the connotation.
The symptoms experienced by patients depend on the organs that are affected 1, 2 when a person has a langerhans cell hisyiocytosis, there is an overproduction and proliferation of langerhans cells. Radiotherapy in langerhans cell histiocytosis a rare. They can be found in the skin, lungs, stomach, bone, eyes and intestines. Symptoms are dyspnea, cough, fatigue, and pleuritic chest pain. Langerhans cell histiocytosis, often called lch, is a disorder where the body produces too many langerhans cells. In this case report, we relate a case of lch affecting the vulva of a 47yearold female. A flow cytometric study of langerhans cell histiocytosis. In langerhans cell histiocytosis, excess immature langerhans cells usually form tumors. Lch cells are a type of dendritic cell which fights infection. Langerhans cell histiocytosis, maxillary sinus introduction langerhans cell histiocytosis lch is a rare disease. Langerhans cell histiocytosis lch treatment may include observation alone, surgery, radiation therapy, or oral, topical, and intravenous medication. Langerhans cell histiocytosis has been known by many names including histocytosis x malignant histiocytosis syndrome now known as t cell lymphoma, eosinophilic.
People with lch produce too many langerhans cells or histiocytes, a form of white blood cell found in healthy people that is supposed to protect the body from infection. The discoveries that these cells are not confined to skin and that they make up a sizable portion of. Langerhans cell histiocytosis lch is one of the histiocytosis disorders, as defined by the histiocyte society. Characterised by the clonal proliferation of pathogenic langerhans cells in single or multiple organs. Texas childrens hospital and baylor college of medicine houston, tx 1. Langerhans cell histiocytosis of the gastrointestinal tract can be a challenging diagnosis to make histologically, particularly when the lesional langerhans cells are obscured by a dense inflammatory cell infiltrate.
Langerhans cell histiocytosis may develop at any age but most commonly occurs in childhood years of age. Langerhans cell histiocytosis lch is a rare disorder in which the body makes too many dendritic cells. 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. Langerhans cell histiocytosis lch is a rare cancer that begins in lch cells. 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. Pulmonary langerhans cell histiocytosis plch is a rare, smokingrelated, lung disease characterized by dendritic cell dc accumulation, bronchiolocentric nodule formation, and cystic lung. In 1868, paul langerhans discovered the epidermal dendritic ce. Langerhans cell histiocytosis lch is a disorder that primarily affects children, but is also found in adults of all ages. Langerhans cell histiocytosis has a highly variable clinical presentation, ranging from a single lesion to potentially fatal disseminated disease. In the rare disease langerhans cell histiocytosis lch, an excess of cells similar to these cells are produced.
Lc aggregates are accompanied by eosinophils, foamy cells, neutrophils. Clinical presentation includes osteolysis, ulcerations of skin and soft tissues but also involvement of the cns is described. This name was agreed upon to recognize the central role of the langerhans cell. In people with lch, these cells multiply excessively and build up in certain areas of the body. Diagnosis is based on history and imaging tests and sometimes on bronchoalveolar. Lch is an abnormal proliferation and dissemination of clonal langerhans cells in which they accumulate, along with other inflammatory cells, and form tissue granulomas in. Langerhans cell histiocytosis is a rare disease that includes a wide range of clinical manifestations. Immunohistochemistry is essential to reach the correct diagnosis. In recent years, treatments developed from our new understanding of the molecular biology of malignant disease have been applied to. Lch is a disease of abnormal clonal proliferation of a unique type of cell in the monocytemacrophage cell line known as the langerhans cell.
Although langerhans cell histiocytosis lch is a familiar entity to. A flow cytometric fcm study of a single case has, however, been published which claimed to provide evidence to contradict this. A langerhans cell is a type of white blood cell that normally helps the body fight off infection. Treatment depends on the site and extent of disease. Multiple organs and systems may be involved by the disease. While langerhans cells are found in normal, healthy people, there is an overproduction and buildup of these cells which can. Langerhans cell histiocytosis lch is a rare multisystem disease with a wide and heterogeneous clinical spectrum and variable extent of involvement. Langerhans cell histiocytosis clinical presentation. Langerhans cell histiocytosis lch is a rare disease marked by proliferation of langerhanstype cells that share immunophenotypic and ultrastructural similarities with antigenpresenting langerhans cells of mucosal sites and skin. Pulmonary langerhans cell histiocytosis plch is proliferation of monoclonal langerhans cells in lung interstitium and airspaces. Langerhans cell histiocytosis lch is a rare, cancerlike condition. Cns involvement in langerhans cell histiocytosis lch is a rare but potentially devastating disorder. Langerhans cell histiocytosis is a rare disease that occurs when normal langerhans cells lose their ability to fight infection and instead group together to destroy healthy tissue. These include mutations of the braf, map2k1, ras and.
1258 1168 761 470 1143 1556 58 1231 1424 483 1168 1280 1247 1333 507 1227 369 884 1499 1053 1434 1098 1244 1204 81 1453 1425 666 176 714 866 1489