You may not embed one of our images on your web page without a link back to our site. Langerhans cell histiocytosis skeletal manifestations. Bohn, julie teruyafeldstein and sergio sanchezsosa july 3rd 20. The highly characteristic common and rare cytological. Pulmonary langerhans cell histiocytosis and other pulmonary histiocytic diseases. Langerhans cell histiocytosis is a rare disease with a variable clinical presentation, caused by monoclonal langerhans cell proliferation. Langerhans cell histiocytosis histiocytosis x guide.
These cells play a role in the bodys immune system. Apr 16, 2010 i would like to report to you the great success i have had with my type 2 diabetes. Please enter your email address to continue to the johns hopkins surgical pathology case. The skeleton is the most commonly involved organ system in langerhans cell histiocytosis lch and is by far the most common location for singlelesion lch, often referred to as eosinophilic granuloma eg the terms are used interchangeably in this article. Selected aspects of the histopathology of langerhans cell histiocytosis representing diagnostic difficulty andor controversy are presented with emphasis on the composition of pathological lesions.
Uncontrolled clonal proliferatoin of langerhans cells. Langerhans cell histiocytosis childrens hospital of. Langerhans cell histiocytosis previously called histiocytosis x involving the skin is the main differential diagnosis of juvenile xanthogranuloma. Aug 20, 2010 the median amount of langerhans cell histiocytosis versus rosaidorfman disease involvement was 7. Department of dermatology, venerology and allergology, kantonsspital st. In the rare disease langerhans cell histiocytosis lch, an excess of cells similar to these cells are produced. While the overwhelming majority of patients are smokers, mechanisms by. Lesional cell phenotypes and the factors influencing variations are noted.
Langerhans cell histiocytosis is a rare condition, slightly more common in boys than girls, affecting around one in 5 million children and fewer adults. For a general discussion of this disease please refer to the article on langerhans cell histiocytosis lch. In bone this may cause pain and adjacent softtissue swelling, but. Jan 22, 2020 langerhans cell histiocytosis is rare, affecting one in 250,000 children and one in 1 million adults in the united states. 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. The nonlch consist of a long list of diverse disorders which have been dif. Langerhans cell histiocytosis and langerhans cell sarcoma. Langerhans cell histiocytosis lch is a group of rare disorders that overproduce and accumulate certain types of white blood cells histiocytes in organs and tissues in the body. Pulmonary langerhans cell histiocytosis in cats and a. Langerhans cell histiocytosis, tongue langerhans cell histiocytosis, tongue. Rooks textbook of dermatology is the most comprehensive work of reference available to the dermatologist. A collection of surgical pathology images langerhans cell histiocytosis histiocytosis x high quality pathology images of orthopedic, tumorlike lesions of bone, langerhans cell histiocytosis. Langerhans cell histiocytosis lch, also designated as histiocytosisx or. Solitary or multiple lesions papules, nodules, plaques containing langerhans cells.
Its prognosis and treatment depend on the extent and severity of disease. The nature of lch and lcs has been actively discussed as exemplified by the title of an article, the langerhans cell histiocytosis x files revealed1 table 1. Lch is part of a group of syndromes called histiocytoses, which are characterized. Langerhans cell histiocytosis, cytological findings. Langerhans cell histiocytosis lch is manifested in a variety of ways, the most common being the eosinophilic granuloma, a localized, often solitary bone lesion that occurs predominantly in the pediatric age group. 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. It used to known as eosinophilic granuloma it has been referred to by several eponyms handschullerchristian disease, abtletterersiwe disease or letterersiwe disease, and histiocytosis x. If you would like a large, unwatermarked image for your web page or blog, please purchase the appropriate license. Bone marrow assessment in langerhans cell histiocytosis. Covering all aspects of skin disease from basic science through pathology and epidemiology to clinical practice, the text is recognized for its unparalleled coverage of diagnosis. Sometimes there are mutations changes in lch cells as they form.
Langerhans cells lcs were first described by paul langerhans, in 1868, as dendritically shaped cells, which were located in the squamous epithelia of epidermis. Langerhans cells are derived from bone marrow, circulate freely from skin to regional lymph nodes in infants, clinically resembles seborrheic keratosis interleukin17 il17 from lesional dendritic cells may drive langerhans cell proliferation nat med 2008. 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. Some forms of histiocytosis resolve without treatment, but others may have a fatal outcome. Langerhans cell sarcoma arising from langerhans cell. When histiocytomas occur as multiple lesions in skin with optional metastasis to lymph nodes and internal organs, the disease resembles cutaneous langerhans cell histiocytosis of humans. Although she did not have a bone survey, this was clinically lymph node limited langerhans cell histiocytosis. Langerhans cell granulomatosis family practice notebook. Langerhans cell histiocytosis lch, previously called histiocytosis x, refers to a spectrum of disease characterized by idiopathic proliferation of histiocytes producing focal or systemic manifestations.
Langerhans cell histiocytosis american society of hematology. Langerhans cell histiocytosis lch is a rare disease of unknown etiology that is characterized by the accumulation of cd1apositive langerhans like cells in involved tissues, which are often. Although it can strike at any age, it is most common in children and young adults, with about 70% of cases occurring before age 17. It primarily occurs in older children and young adults, with a male to female ratio of 2. Langerhans cell histiocytosis lch is a disorder that primarily affects children, but is also found in adults of all ages. Recent evidence suggests that it is a neoplastic proliferation potentially derived from amyeloidlineage precursor. Clinical significance langerhans cell histiocytosis. Composed of langerhans cells uniform, bland nuclei pale chromatin, thin nuclear membrane, inconspicuous nucleoli. As opposed to juvenile xanthogranuloma, cells in the former condition are more spindly, with coffeebean nuclei. However lch cells stain positive to cd14 which is a monocyte marker and shows a different, hematopoietic origin for the disorder. Langerhans cells are normally present as a few cells in thymus, lymph nodes and skin.
Langerhans cell histiocytosis may develop at any age but most commonly occurs in childhood years of age. Examples of multiuser license include books, book chapters, educational material, and other publications with multiple copies. Langerhans cells lc are bone marrowderived antigen presenting cells 1. Either term refers to three separate illnesses listed in order of increasing severity. Langerhans cell histiocytoses rooks textbook of dermatology. Langerhans cell histiocytosis lch is a rare disorder in which the body makes too many dendritic cells. These include mutations of the braf, map2k1, ras and. Langerhans cell histiocytosis lch is manifested in a variety of ways, the most common being the eosinophilic granuloma, a localized, often solitary bone lesion that occurs predominantly in the. Langerhans cell histiocytosis lch and lymphangioleiomyomatosis lam duration.
Pulmonary langerhans cell histiocytosis orphanet journal of. While langerhans cells are found in normal, healthy people, there is an overproduction and buildup of these cells which can. The french langerhans cell histiocytosis study group 1996 a multicenter retrospective survey of langerhans cell histiocytosis. We provide 1 original jpeg or tiff and 1 highquality optimized image without our logo. Ultrastructurally, the birbeck or x granule is straight or curved pentalaminary rod of variable length, often vesicular at one end frequently resembling a racquet. The histopathology of langerhans cell histiocytosis. Maarten egeler langerhans cell histiocytosis in adults. Lch is part of a group of syndromes called histiocytoses, which are characterized by an abnormal proliferation of histiocytes an. In children younger than 2 years of age, cutaneous involvement is the most common presentation. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment.
Research that demonstrated langerhans cell involvement as well as histiocytes led to a proposal that the term langerhans cell histiocytosis lch be used in place of histiocytosis x. Langerhans cell histiocytosis lch is a rare hematological disorder characterized by local or generalized, uncontrolled proliferation and infiltration of langerhans type of histiocytic cells. Radiation oncologyhistiocytosis wikibooks, open books. The skeletal system is the most common site of langerhans cell histiocytosis involvement, and in 6080% of cases is the only organ system involved. Langerhans cell histiocytosis is a clonal hematopoietic disorder characterized by proliferation of langerhans cells in one or more organs. Noah brown, md langerhans cell histiocytosis lch comprises a wide spectrum of clinical disorders that have in common a proliferation of langerhans type cells with characteristic morphologic, immunophenotypic, and ultrastructural. Oral surg oral med oral pathol oral radiol endod 2005.
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 atlas of genetics and. Individualized treatment approaches for langerhans cell. Department of pathology, university of miami, miami, fl. In people with lch, these cells multiply excessively and build up in certain areas of the body. The hallmark of lch is the proliferation and accumulation of a specific histiocyte. Pathology of langerhans cell histiocytosis histiocytosis x. The cytology of langerhans cell histiocytosis histiocytosis. 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. Langerhans cell histiocytosis radiology reference article. Pulmonary langerhans cell histiocytosis springerlink. Occasionally and confusingly, the term histiocytosis is sometimes used to refer to individual diseases. Percutaneous techniques for the diagnosis and treatment of localized langerhans cell histiocytosis eosinophilic granuloma of bone.
Adult lch patients are often evaluated by physicians who focus on only the most obviously affected organ without sufficient evaluation of. Nosology and pathology of langerhans cell histiocytosis. This page includes the following topics and synonyms. The cytomorphology of cases of langerhans cell histiocytosis is described. Later on, these cells were identified in all stratified squamous epithelium of mammals. Langerhans cell histiocytosis in an adult with oral cavity. Langerhans cells capture antigens and present them to lymphocytes. Langerhans cell histiocytosis genetic and rare diseases. However, recent studies suggest abnormal immune regulation as an important factor. Langerhans cell histiocytosis lch is a rare disorder.
Pulmonary langerhans cell histiocytosis surgical pathology criteria. Skin biopsy diagnosis of langerhans cell neoplasms. The complexity of the lesions was due largely to the absence of data regarding clonality and genetic. Symptoms range from isolated bone lesions to multisystem disease. Pulmonary langerhans cell histiocytosis orphanet journal of rare.
Lch can cause damage to skin, bone and other organs hiv. Dendritic cells dcs play an important role in local defense mechanisms in the epithelium. According to the histiocytosis association of america, 1 in 200,000 children in the united. Diagnosis of langerhans cell histiocytosis on fine needle. It represents a spectrum of clinicopathologic disorders, ranging from a highly aggressive and frequently fatal multisystem disease to an easily cured. Characterized by the clonal proliferation of pathogenic langerhans cells in single or multiple organs.
Tumorlike lesions of bone of langerhans cell histiocytosis. 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. 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. Lch cells are a type of dendritic cell which fights infection. In neonates and young infants, skin is frequently involved, as solitary or multiple papules or nodules with ulceration or necrosis. Granulocytes, lymphocytes, and eosinophils are always found figs.
An overview of a more recent classification is found in the article classification of histiocytoses and neoplasms of the macrophagedendritic cell lineages langerhans cell histiocytosis. Langerhans cell histiocytosis high quality pathology images of orthopedic. Dendritic cells are a form of histiocyte, or white blood cell. A case report langerhans cell sarcoma lcs is a neoplastic proliferation of langerhans cells that have overtly malignant cytologic features. Jul 28, 2004 langerhans cell histiocytosis lch is manifested in a variety of ways, the most common being the eosinophilic granuloma, a localized, often solitary bone lesion that occurs predominantly in the pediatric age group. During childhood, langerhans cell histiocytosis peaks between ages 1 and 3. A case of multifocal langerhans cell histiocytosis in a twoyearold child is presented where fine needle aspiration was helpful in achieving a rapid and accurate diagnosis in an appropriate clinical and radiological setting. This can avoid unnecessary biopsy and guide the management especially where access to histopathology is limited. Fascin, a 55kd actinbundling protein, represents a highly selective marker for dendritic cells of lymphoid tissues and peripheral blood and is involved in the formation of dendritic processes in maturing epidermal langerhans cells. Cutaneous histiocytoses are classified according to the type of histiocyte within the skin. This book is distributed under the terms of the creative commons attribution 4. Pulmonary langerhans cell histiocytosis surgical pathology.
The newer term is preferred as its more descriptive of its cellular background, and removes the ambiguity of the connotation. This uptodate and comprehensive dvdrom disc provides a superb collection of authoritative documents from the nations cancer experts on childhood langerhans cell histiocytosis lch histiocytosis x, eosinophilic granuloma, abtletterersiwe, handschullerchristian, diffuse reticuloendotheliosis. Johns hopkins surgical pathology unknown conference. Langerhans cell histiocytosis genetics home reference nih. Jaffe r 2011 langerhans cell histiocytosis and langerhans cell sarcoma. Skin biopsy diagnosis of langerhans cell neoplasms, skin biopsy diagnosis and treatment, suran l. Lch is generally regarded as a sporadic disease that occurs predominantly in the paediatric population. Langerhans cell sarcoma arising from langerhans cell histiocytosis. They can be found in the skin, lungs, stomach, bone, eyes and intestines.
In medicine, histiocytosis is an excessive number of histiocytes tissue macrophages, and the term is also often used to refer to a group of rare diseases which share this sign as a characteristic. It has been referred to by several eponyms handschullerchristian disease, abtletterersiwe disease or letterersiwe disease, and histiocytosis x. Sep 11, 2017 langerhans cell histiocytosis lch is a disorder that primarily affects children, but is also found in adults of all ages. Langerhans cell histiocytosis archives of pathology. Within a month of listening to you, i managed to reduce my doses of medication between 50 and 80%. Focused langerhans cell histiocytosis with stained slides of pathology. Isolated langerhans cell histiocytosis of the stomach. Langerhans cell histiocytosis lch is currently regarded as a myeloid neoplasm, with remarkably broad clinical spectrum, ranging from isolated skin or bone lesions to a disseminated disease that can involve nearly any organ.
Pulmonary langerhans cell histiocytosis plch is a relatively. It broadly fits into the category of histiocytoses. Most of the knowledge about the diagnosis and therapy is based on pedriatic studies. Langerhans cell histiocytosis lch is a rare multisystem disease with a wide and heterogeneous clinical spectrum and variable extent of involvement. Langerhans cell lc histiocytosis lch and lc sarcoma lcs constitute proliferative disorders of lcs. Langerhans cell histiocytosis was previously known as histiocytosis x. Langerhans cell histiocytosis statpearls ncbi bookshelf. Infiltration by langerhans cells polygonal cells with eosinophilic cytoplasm, oval nuclei with longitudinal grooves resembling coffee beans eosinophils, giant cells, neutrophils, foam cells, lymphocytes, plasma cells, fibrosis, necrosis. Langerhans cell histiocytosis lch is a clonal disorder believed to be derived from cells of the dendritic system.
Langerhans cell histiocytosis etiology bmj best practice. Langerhans cell histiocytosis lch is a rare cancer that begins in lch cells. Langerhans cell histiocytosis, abbreviated lch, is a rare disorder of tissue macrophages. Pulmonary langerhans cell histiocytosis plch is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. Skin biopsy diagnosis of langerhans cell neoplasms intechopen. Pathology multiple centrilobular, discrete nodular infiltrates peribronchiolar with stellate extension into surrounding alveolar walls. Although it is generally agreed that pathologic langerhans cells play a. Canine cutaneous histiocytoma originates from langerhans cells lcs indicated by expression of cd1a, cd11ccd18, and ecadherin. Langerhans cell histiocytosis lch and lymphangioleiomyomatosis lam medicosis perfectionalis. The tumor cells of langerhans cell histiocytosis have a rich grayblue cytoplasm with a groovedindented nucleus with fine chromatin. Lch can affect a wide range of patients, including neonates, young children and adults.
Em electron microscopy the classification has evolved considerably, as the entities can overlap. The langerhans cell displays immunoreactivity cytoplasmic and nuclear for s100 protein and the monoclonal antibody cd1a, hladr ia and peanut agglutinin. Langerhans cell histiocytosis lch is an orphan disease of clonal dendritic cells which may affect any organ of the body. 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 an overview sciencedirect. Langerhans cell histiocytosis is a disorder in which excess immune system cells called langerhans cells build up in the body. Langerhans cell histiocytosis johns hopkins pathology.
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