• Langerhans cell histiocytosis is a rare disease involving clonal proliferation of langerhans cells seen in children and young adults. (scirp.org)
  • The Langerhans Cell Histiocytosis (LCH) is a disease characterized by the clonal proliferation of Langerhans cells. (authorea.com)
  • Langerhans cell histiocytosis (LCH) is an idiopathic group of reactive proliferative diseases linked to aberrant immunity, pathologically characterized by clonal proliferation of Langerhans cells. (engineering.org.cn)
  • Langerhans' cell histiocytosis (LCH), previously known as histiocytosis X, describes a rare spectrum of conditions with abnormal proliferation of histiocytes, occuring in various locations throughout the body. (reviewofophthalmology.com)
  • Microscopic review of the tissue specimen revealed proliferation of histiocytes (left) with positive CD1a staining, a marker for Langerhans' cells (right). (reviewofophthalmology.com)
  • Histiocytosis is an umbrella term for an emerging spectrum of "syndromes" or disorders that involve abnormal proliferation of specialized white blood cells (histiocytes). (petmoo.com)
  • In general, Histiocytosis refers to an abnormally large amount of histiocytes. (petmoo.com)
  • A cutaneous histiocytoma is an abnormal proliferation of histiocytes in Langerhans cells. (petmoo.com)
  • Idiopathic non-malignant disease characterized by idiopathic infiltration and accumulation of abnormal histiocytes (i.e. the Langerhans cells) within various tissues (bone marrow, skin, central nervous system, lung, liver, spleen, lymph nodes) causing focal or systemic effects. (capsulehealth.one)
  • This mutation leads to production of a BRAF protein that is abnormally active, which disrupts regulation of cell proliferation and may allow histiocytes to grow and divide uncontrollably, leading to the abnormal accumulation of histiocytes that occurs in Erdheim-Chester disease. (medlineplus.gov)
  • Benign disorder of infants and children caused by proliferation of HISTIOCYTES , macrophages found in tissues. (nih.gov)
  • These histiocytes, usually lipid-laden non-Langerhans cells, form multiple yellow-red nodules most often in the skin, the eye, and sometimes in the viscera. (nih.gov)
  • This disease is characterized by enhanced proliferation (reproduction of cellular elements) of histiocytes, as well as their accumulation in different tissues and the formation of granulomas. (antibiotic-store.com)
  • Virus-associated hemophagocytic syndrome (VAHS) is a fulminant disorder associated with systemic viral infection and is characterized pathologically by the proliferation of hemophagocytic histiocytes in the lymphoreticular tissues. (cdc.gov)
  • Juvenile xanthogranuloma (JXG) is a benign, self-limiting reactive prolif- erative disorder of non-Langerhans cell histiocytes seen predominantly in infants and young children and occa- sional y in adults. (who.int)
  • Pulmonary Langerhans cell histiocytosis (PLCH) is proliferation of monoclonal Langerhans cells in lung interstitium and airspaces. (msdmanuals.com)
  • Pulmonary Langerhans cell histiocytosis is a disease in which monoclonal CD1a-positive Langerhans cells (a type of histiocyte) infiltrate the bronchioles and alveolar interstitium, accompanied by lymphocytes, plasma cells, neutrophils, and eosinophils. (msdmanuals.com)
  • Effectiveness of cladribine therapy in patients with pulmonary Langerhans cell histiocytosis. (unil.ch)
  • Two decades later, with the advent of electron microscopy, Nezelof and colleagues identified a unique intracellular organelle, the Birbeck granule , in histiocytosis X lesions. (capsulehealth.one)
  • 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. (thedoctorsdoctor.com)
  • It is impossible to avoid skin lesions with a generalized form of histiocytosis X. Most often the skin is affected in children. (antibiotic-store.com)
  • This work represents an important advance in understanding how the lesions of Langerhans Cell Histiocytosis develop. (histiouk.org)
  • Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the presence of cells with characteristics similar to bone marrow-derived Langerhans cells juxtaposed against a backdrop of hematopoietic cells, including T-cells, macrophages, and eosinophils. (medscape.com)
  • Pathology of the tissue biopsy demonstrated proliferation of macrophages or dendritic cells, and positive staining with CD1a confirmed a diagnosis of Langerhans' cell histiocytosis (See Figure 2) . (reviewofophthalmology.com)
  • Pathophysiology may involve recruitment and proliferation of Langerhans cells in response to cytokines and growth factors secreted by alveolar macrophages in response to cigarette smoke. (msdmanuals.com)
  • [16] One theory proposed the coexistence of LCH and JXG could be the result of chemotherapy-induced maturation of Langerhans' cells into macrophages, in particular foamy cells. (eyewiki.org)
  • Hemophagocytic lymphohistiocytosis (HLH) is characterized by aberrant activation and proliferation of polyclonal CD8 + T lymphocytes and macrophages that infiltrate multiple organs and overproduce inflammatory cytokines [ 1 ]. (biomedcentral.com)
  • 1.2/ Investigate the development and functions of 'resident' networks of macrophages and Dcs such as langerhans cells and microglia, and their potential roles in inflammation. (cerclefser.org)
  • The working group of the Histiocyte Society divided histocytic disorders into three groups: (1) dendritic cell histiocytosis, (2) macrophage-related disorders, and (3) malignant histiocytosis. (medscape.com)
  • More recently, histiocytic diseases have been reclassified into five groups: (1) Langerhans-related, (2) cutaneous and mucocutaneous, (3) malignant histiocytosis, (4) Rosai-Dorfman disease, and (5) hemophagocytic lymphohistiocytosis and macrophage activation syndrome. (medscape.com)
  • M group: Includes Malignant Histiocytosis. (petmoo.com)
  • Malignant histiocytic diseases include malignant histiocytosis (now it is called localized and disseminated histiocytic sarcoma) and cutaneous histiocytoma, which is a benign histiocytic tumor. (petmoo.com)
  • Treatment protocol for malignant histiocytosis is directed towards controlling the local tumor and tackling the concern for metastasis. (petmoo.com)
  • Malignant histiocytosis cannot be completely cured even with medical management. (petmoo.com)
  • Malignant Histiocytosis is a potentially life-threatening disease in dogs but the mortality rate of dogs is not documented. (petmoo.com)
  • Malignant histiocytosis prognosis for recovery is poor. (petmoo.com)
  • Some researchers believe that langerhans cell histiocytosis in children is a malignant disease, while others consider this form of histiocytosis as a non-tumor process. (antibiotic-store.com)
  • With malignant histiocytosis, a high mortality rate. (antibiotic-store.com)
  • OBJECTIVE: The etiology and pathogenesis of Langerhans cell histiocytosis (LCH) remain poorly understood. (thedoctorsdoctor.com)
  • Nonmalignant Nonneoplastic - Reactive (cutaneous or systemic) histiocytosis. (petmoo.com)
  • cell histiocytosis characterized by for evaluation of an asymptomatic skin Complete cutaneous examination re- accumulation of lipid-laden macro- lesion on the penile shaft that has been vealed 10 variably sized CALMs rang- phages. (who.int)
  • Notably, LCH cells have been found to express markers of both resting epidermal Langerhans cells (CD1a, intracellular major histocompatibility complex II [MHCII], Birbeck granules) and activated Langerhans cells (including CD54 and CD58). (medscape.com)
  • The serine/threonine protein kinase BRAF is an important player in the mitogen-activated protein kinase (MAPK) signaling pathway that transduces mitogenic signals from activated cell-surface growth factor receptors to the cell nucleus and as a result modulates many important cellular processes, such as tumor growth, differentiation, proliferation, and angiogenesis. (hindawi.com)
  • The pathogenesis of Langerhans cell histiocytosis (LCH) is unknown. (medscape.com)
  • We report a rare case of adult Langerhans cell histiocytosis in the dorsal spine causing a spinal cord compression associated with a pulmonary process treated by surgery, radiotherapy and systemic therapy with good evolution. (scirp.org)
  • Histiocytosis can be both a systemic neoplasm, and can be expressed in the formation of benign single granulomas. (antibiotic-store.com)
  • The spectrum of non-langerhans cell histiocytoses include: Benign cephalic histiocytosis Generalized eruptive histiocytoma Indeterminate cell histiocytosis Juvenile xanthogranuloma Progressive nodular histiocytoma Necrobiotic xanthogranuloma (Giant Cell) Reticulohistiocytoma Multicentric reticulohistiocytosis Rosai-Dorfman disease Xanthoma disseminatum Kikuchi disease Erdheim-Chester disease. (wikipedia.org)
  • Juvenile xanthogranuloma (JXG) is a rare benign histiocytic proliferation that develops in infants and young children. (eyewiki.org)
  • benign, self-healing, non-Langerhans was referred to the dermatology clinic No dysmorphic features were noted. (who.int)
  • Epidermal Langerhans cells are unique among dendritic cells in that they arise not from myeloid progenitor cells in bone marrow but rather from yolk-sac progenitors and fetal liver-derived monocytes that populate the skin before birth and are maintained locally under steady-state conditions. (capsulehealth.one)
  • In 1868, Paul Langerhans discovered the epidermal dendritic cells that now bear his name. (medscape.com)
  • Langerhans cells are named after Paul Langerhans , a bright, young medical student who worked with the new technique of gold colloid staining in the mid-19th century. (capsulehealth.one)
  • The Langerhans cell is a dendritic cell, found in many organs. (springeropen.com)
  • [ 3 ] Taken together, these findings have led some to speculate that LCH is not a specific disease of epidermal Langerhans cells, but rather one of mononuclear phagocyte dysregulation. (medscape.com)
  • Langerhans cell histiocytosis (LCH) is a disease characterized by the abnormal proliferation of leukocytes in the liver, lung and spleen. (histio.org)
  • Rosai-Dorfman disease is a rare disorder characterized by overproduction (proliferation) and accumulation of a specific type of white blood cell (histiocyte) in the lymph nodes of the body (lymphadenopathy), most often those of the neck (cervical lymphadenopathy). (rarediseases.org)
  • Symptoms of Langerhans' Cell Histiocytosis vary depending on the part of the body affected and the severity of the disease. (homeremedylifestyle.com)
  • Langerhans' cell histiocytosis is a rare disease . (homeremedylifestyle.com)
  • The best way to treat Langerhans' cell histiocytosis is to have the disease treated by experts who understand the disease. (homeremedylifestyle.com)
  • L group: Erdheim-Chester Disease, Langerhans Cell Histiocytosis, etc. (petmoo.com)
  • Overview of Interstitial Lung Disease Interstitial lung diseases are a heterogeneous group of disorders characterized by alveolar septal thickening, fibroblast proliferation, collagen deposition, and, if the process remains unchecked. (msdmanuals.com)
  • Langerhans cell histiocytosis (LCH) is a rare disease that involves the abnormal proliferation of Langerhans cells in the skin. (osmosis.org)
  • 10.5137/1019-5149.JTN.10542-14.1 Rosai-Dorfman Disease involves histiocytic proliferation of the lymphatic system. (turkishneurosurgery.org.tr)
  • Furthermore, the combination of this disease entity with Langerhans cell histiocytosis is an even rarer phenomenon that has only recently been highlighted. (turkishneurosurgery.org.tr)
  • This is only the second report of the co-occurrence of Langerhans histiocytosis and Rosai-Dorfman disease in the cranium and intracranial cavity. (turkishneurosurgery.org.tr)
  • Hand - Schuller - Christian disease is a variant of histiocytosis that is also rare. (antibiotic-store.com)
  • Pneumofibrosis is a disease characterized by proliferation of the connective tissue of the lung due to the inflammatory process, which causes a violation of the structure of the lungs and a decrease in their ventilation function. (antibiotic-store.com)
  • Other disorders similar to LCH include Histiocytoses Rosai-Dorfman, juvenile xanthogranuloma, and Erdheim-Chester disease but they do not share the exact phenotypic signature of LCH. (netlify.app)
  • The abnormal proliferation and formation of cysts, causes obstructive pattern of lung disease [9] . (wikidoc.org)
  • JXG does not carry BRAF V600E mutation, except in patients with both Langerhans' cells histiocytosis (LCH) and JXG (non-Langerhan cells histiocytosis) diseases and in the aggressive forms of JXG. (eyewiki.org)
  • Histiocytosis in children is a group of diseases that are quite rare in medical practice, proceed in different ways, but are combined by proliferative processes in the monocytic-macrophage system. (antibiotic-store.com)
  • Histiocytosis UK is dedicated to promoting and funding scientific research into uncovering not only the causes of all histiocytic diseases, which include Langerhans's Cell Histiocytosis and Haemophagocytic Lymphohistiocytosis, but also ensuring early diagnosis, effective treatment and a cure. (histiouk.org)
  • However, it is not only a powerful mechanism to control proliferation of microorganisms, it also -unfortunately- damages host tissues and contributes in the long term to many features of ageing and to cardiovascular and neurological diseases. (cerclefser.org)
  • They are a critical mediator of the inflammatory response, which is a powerful mechanism to control proliferation of microorganisms, but that also -unfortunately- damages host tissues and contribute in the long term to many features of ageing and to cardiovascular and neurological diseases that decrease quality of life and shorten our lifespan. (cerclefser.org)
  • 2009), and investigated the pathophysiology of diseases of this cellular system, incliding Langerhans cell histiocytosis (Senechal et al. (cerclefser.org)
  • Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is characterized by multifocal proliferation of pulmonary neuroendocrine cells. (biomedcentral.com)
  • Positive immunohistological staining with neuronal markers S100 and CD1a are specific for Langerhans' cells and help to confirm the diagnosis. (reviewofophthalmology.com)
  • LCH is characterized by the proliferation of CD1a-positive activated Langerhans (not atypical Langerhans cell, morphologically)-like cells (LCH cells) generating inflammatory granuloma. (mayabouchenaki.com)
  • The histological (associated with the microscopic structure of tissues) manifestations of various clinical variants of histiocytosis in their course do not have particularly significant differences. (antibiotic-store.com)
  • Erdem AP, Kasimoglu Y, Sepet E, Gencay K, Sahin S, Dervisoglu S. Oral manifestations may be the first sign of Langerhans cell histiocytosis. (mayabouchenaki.com)
  • Langerhans Cell Histiocytosis Langerhans cell histiocytosis (LCH) is a proliferation of dendritic mononuclear cells with infiltration into organs locally or diffusely. (msdmanuals.com)
  • At this point, Birbeck granules were thought to be exclusive to epidermal Langerhans cells, skin-restricted cells of the mononuclear phagocyte system. (capsulehealth.one)
  • The antibody is a new monoclonal antibody against a C-type lectin found on the surface of Langerhans cells. (homeremedylifestyle.com)
  • To date, it has been proven that the proliferation process (neoplasm of cells and intracellular structures - mitochondria, endoplasmic reticulum, ribosomes, etc.) of Langerhans cells with histiocytosis X is characterized by a monoclonal (belonging to the same cell clone) character. (antibiotic-store.com)
  • A young male, who had previously undergone surgical excision of a skull lesion that was reported as Langerhans histiocytosis presented with an intracranial lesion mimicking a meningioma. (turkishneurosurgery.org.tr)
  • Final diagnosis was therefore Langerhans cell histiocytosis of the dorsal spine complicated by spinal cord compression. (scirp.org)
  • Having a diagnosis of Langerhans' Cell Histiocytosis can be a scary time for a family. (homeremedylifestyle.com)
  • A diagnosis of Langerhans' Cell Histiocytosis is dependent on a number of factors. (homeremedylifestyle.com)
  • A diagnosis of Langerhans' cell histiocytosis should be made only after the patient has undergone a biopsy . (homeremedylifestyle.com)
  • Lichtenstein proposed a common diagnosis, histiocytosis X , with the X indicating an uncertain cell of origin. (capsulehealth.one)
  • The histopathologic diagnosis of the lingual neoformation was histiocitary proliferation with the presence of giant cells, Touton type, compatible with juvenile xanthogranuloma. (biomedcentral.com)
  • Over the next decades, reviews debated whether LCH was a disorder of transformed Langerhans cells or of normal Langerhans cells rendered pathologic by inappropriate stimuli. (capsulehealth.one)
  • In a normal physiological, "non-dangerous" situation, LCs coordinate a continuous state of immune tolerance Langerhans cell histiocytosis (LCH) is a rare disorder in which the body makes too many dendritic cells. (netlify.app)
  • Histiocyte cells are a form of white blood cells that help the immune system destroy Langerhans cell histiocytosis is a rare disorder that can look like some types of cancer. (netlify.app)
  • Sinus histiocytosis with massive lymphadenopathy (SHML), initially described in 1969 by Rosai and Dorfman,[ 22 ] is a rare, nonneoplastic lymphoproliferative disorder that is characterized by its histological features. (surgicalneurologyint.com)
  • Lymphangiomyomatosis is a disorder resulting from proliferation of abnormal smooth muscle like cells, mostly in the lungs but can also occur in other body parts such as kidney , mediastinum or axial lymphatics . (wikidoc.org)
  • In 1868 , Langerhans described an epidermal cell population, accounting for approximately 1% of epidermal cells, with characteristic dendrites that he described as extracutaneous nerves. (capsulehealth.one)
  • Langerhans cell histiocytosis (LCH) and In LCH, overproduced, immature Langerhans cells cluster together and Cytokine pattern of Langerhans cells isolated from murine epidermal cell cultures. (netlify.app)
  • We present a case of multiorgan, multisite Langerhans cell histiocytosis (LCH), positive for the oncogenic BRAF V600E mutation, in a preterm neonate. (thieme-connect.de)
  • Recurrent BRAF mutations in Langerhans cell histiocytosis. (wanfangdata.com.cn)
  • Juvenile xanthogranuloma is a rare non-Langerhans cell histiocytosis that usually occurs during infancy and early childhood. (biomedcentral.com)
  • The ultrastructural hallmark of the Langerhans cell, the Birbeck granule, was described a century later. (medscape.com)
  • Specifically, a variety of other cellular populations have been identified that possess phenotypic characteristics similar to Langerhans cells, including expression of CD207 and Birbeck granules. (medscape.com)
  • Langerhans cell histiocytoses are all defined by the presence of a unique ultrastructural organelle, the Birbeck granule. (thedoctorsdoctor.com)
  • The term Langerhans cell histiocytosis is generally preferred to the older term, histiocytosis X. This newer name emphasizes the histogenesis of the condition by specifying the type of lesional cell and removes the connotation of the unknown ("X") because its cellular basis has now been clarified. (medscape.com)
  • Therefore, in addition to epidermal Langerhans cells, other potential cellular origins for LCH include dermal langerin + dendritic cells, lymphoid tissue-resident langerin + dendritic cells, and monocytes that can be induced by local environmental stimuli to acquire a Langerhans cell phenotype. (medscape.com)
  • One of the key cellular components with functional roles in both innate and adaptive arms of the immune response are Langerhans cells (LCs) (2). (mayabouchenaki.com)
  • Clinical and microbiological evaluation of the oral cavity of patients with Langerhans Cell Histiocytosis. (authorea.com)
  • Patients appear to have normal lipid metabolism and are classified as a normolipemic non-Langerhans cell histiocytosis. (nih.gov)
  • When asked "What does it mean for our paediatric patients of Langerhans Cell Histiocytosis? (histiouk.org)
  • Langerhans cells and melanocytes are connected to adjacent cells by desmosomes the same way keratinocytes are connected to one another. (netlify.app)
  • A chest CT scan revealed a tumoral process measuring 25 mm at the lower lobe of the left lung evoking a Langerhans cell histiocytosis ( Figure 3 ). (scirp.org)
  • It is characterized by an interstitial proliferation of Langerhans cells in the lung parenchyma. (nih.gov)
  • Excessive proteolytic activity from the proliferation of the smooth muscle cells result in lung destruction and formation of cysts. (wikidoc.org)
  • This proliferation of immature muscle cells starts covering alveolar walls, bronchioles , pleura and vessels, including lymphatic routes. (wikidoc.org)
  • Langerhans' cell histiocytosis can cause symptoms in the skin, bones, lungs, and other parts of the body. (homeremedylifestyle.com)
  • Langerhans cell histiocytosis affecting the lungs. (nih.gov)
  • Available at: http://www.cancernetwork.com/oncology-journal/langerhans-cell-histiocytosis-emerging-insights-and-clinical-implications [Accessed 1 Jul. (capsulehealth.one)
  • This book is about the phenotypic and functional features of epidermal Langerhans cells in experimental models and in clinical medicine. (netlify.app)