• 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)
  • 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)
  • 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)
  • The pathogenesis of Langerhans cell histiocytosis (LCH) is unknown. (medscape.com)
  • Langerhans cell histiocytosis (LCH) is an abnormal clonal proliferation of Langerhans cells, abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes. (wikipedia.org)
  • The disease spectrum results from clonal accumulation and proliferation of cells resembling the epidermal dendritic cells called Langerhans cells, sometimes called dendritic cell histiocytosis. (wikipedia.org)
  • Unifocal LCH, also called eosinophilic granuloma (an older term which is now known to be a misnomer), is a disease characterized by an expanding proliferation of Langerhans cells in one organ, where they cause damage called lesions. (wikipedia.org)
  • This primary bone involvement helps to differentiate eosinophilic granuloma from other forms of Langerhans Cell Histiocytosis (Letterer-Siwe or Hand-Schüller-Christian variant). (wikipedia.org)
  • Pulmonary Langerhans cell histiocytosis (PLCH) is a unique form of LCH in that it occurs almost exclusively in cigarette smokers. (wikipedia.org)
  • PLCH develops when an abundance of monoclonal CD1a-positive Langerhans (immature histiocytes) proliferate the bronchioles and alveolar interstitium, and this flood of histiocytes recruits granulocytes like eosinophils and neutrophils and agranulocytes like lymphocytes further destroying bronchioles and the interstitial alveolar space that can cause damage to the lungs. (wikipedia.org)
  • Broadly speaking, they can be divided into Langerhans and non-Langerhans cell histiocytoses. (thedoctorsdoctor.com)
  • Langerhans cell histiocytoses are all defined by the presence of a unique ultrastructural organelle, the Birbeck granule. (thedoctorsdoctor.com)
  • Currently, the preferred term is Langerhans cell histiocytosis. (thedoctorsdoctor.com)
  • Langerhans cell histiocytosis presenting in the neonatal period: a retrospective case series. (thedoctorsdoctor.com)
  • 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)
  • Epidemiologic study of Langerhans cell histiocytosis in children. (thedoctorsdoctor.com)
  • OBJECTIVE: The etiology and pathogenesis of Langerhans cell histiocytosis (LCH) remain poorly understood. (thedoctorsdoctor.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)
  • Localized histiocytosis is known as eosinophilic granuloma, as well as Langerhans cell granuloma. (antibiotic-store.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)
  • 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)
  • 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)
  • No consensus exists for the optimal therapy for Langerhans cell histiocytosis (LCH), particularly in the case of multisystem organ disease. (medscape.com)
  • Single-agent chemotherapy with cladribine (2-chlorodeoxyadenosine/2-CdA) may be a promising treatment for single-system pulmonary Langerhans cell histiocytosis. (medscape.com)
  • C group: Non-Langerhans Cell Histiocytosis (skin). (petmoo.com)
  • L group: Erdheim-Chester Disease, Langerhans Cell Histiocytosis, etc. (petmoo.com)
  • A cutaneous histiocytoma is an abnormal proliferation of histiocytes in Langerhans cells. (petmoo.com)
  • 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)
  • Patients appear to have normal lipid metabolism and are classified as a normolipemic non-Langerhans cell histiocytosis. (nih.gov)
  • In conclusion, BRAF mutations in histiocytic proliferations are restricted to lesions of the Langerhans-cell type. (oncotarget.com)
  • An International team of Researchers including researchers from Newcastle, Dr Paul Milne and Prof Matthew Collin have made progress in understanding how the childhood cancer Langerhans Cell Histiocytosis (LCH) damages tissue in the body. (histiouk.org)
  • This work represents an important advance in understanding how the lesions of Langerhans Cell Histiocytosis develop. (histiouk.org)
  • When asked "What does it mean for our paediatric patients of Langerhans Cell Histiocytosis? (histiouk.org)
  • Juvenile xanthogranuloma Langerhans cell histiocytosis (LCH) is a proliferation of dendritic mononuclear cells with infiltration into organs locally or diffusely. (msdmanuals.com)
  • 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)
  • Langerhans Cell-Histiocytos Langerhans Cell-Histiocytos (LCH) är en ovanlig och gåtfull sjukdom som i huvudsak drabbar barn och som tidigare varit mest känd under beteckningen Histiocytosis X. Förekomst Enligt vad vi vet idag drabbas 5-10 barn i Sverige av sjukdomen varje år. (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)
  • 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)
  • Here, we outline the most likely diagnoses with this presentation and discuss a case of Langerhans cell histiocytosis (LCH) in a preterm neonate with severe multiorgan involvement. (thieme-connect.de)
  • Langerhans cell histiocytosis is a rare disease involving clonal proliferation of langerhans cells seen in children and young adults. (scirp.org)
  • 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)
  • Final diagnosis was therefore Langerhans cell histiocytosis of the dorsal spine complicated by spinal cord compression. (scirp.org)
  • 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)
  • Effectiveness of cladribine therapy in patients with pulmonary Langerhans cell histiocytosis. (unil.ch)
  • solid course="kwd-title" Keywords: Erdheim Chester Disease, Langerhans cell histiocytosis, Cladribine, Treatment 1. (bioskinrevive.com)
  • Launch Erdheim Chester Disease (ECD) is a rare non-Langerhans cell histiocytosis. (bioskinrevive.com)
  • Cladribine is normally FDA accepted for hairy cell leukemia and provides other off-label uses, including Langerhans cell Histiocytosis (LCH) and various other lymphoproliferative disorders [6]. (bioskinrevive.com)
  • JXG is the most common form of non-Langerhans' cell histiocytoses. (eyewiki.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)
  • 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)
  • Histiocytosis X was renamed Langerhans-cell histiocytosis , reflecting the concept that LCH cells represented dysfunctional epidermal Langerhans cells. (capsulehealth.one)
  • Available at: http://www.cancernetwork.com/oncology-journal/langerhans-cell-histiocytosis-emerging-insights-and-clinical-implications [Accessed 1 Jul. (capsulehealth.one)
  • 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)
  • LCH is part of a group of syndromes called histiocytoses, which are characterized by an abnormal proliferation of histiocytes (an archaic term for activated dendritic cells and macrophages). (wikipedia.org)
  • Benign disorder of infants and children caused by proliferation of HISTIOCYTES , macrophages found in tissues. (nih.gov)
  • Increased interaction-activation of histiocytes (macrophages) and CD8 + T-cells, the key pathogenic feature of MAS, was observed in the glomeruli of this patient, who did not demonstrate overt systemic manifestations, suggesting a pathological condition of renal-limited MAS. (biomedcentral.com)
  • 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)
  • Malignant histiocytosis is a rare invasive proliferation of neoplastic histiocytes. (bmj.com)
  • Even secondary HPS occurs as an imbalance between insufficient host defense, obstinate hyperinflammation, and a heterogeneous triggering event, which can be of infectious, rheumatic or neoplastic nature: therefore, the clinical disease results as the signature of a dysregulated immune activation, leading to macrophage proliferation and widespread hemophagocytosis in the reticuloendothelial system. (biomedcentral.com)
  • The nodules resemble to some extent the GERMINAL CENTER of lymph node follicles and most likely represent neoplastic proliferation of lymph node-derived follicular center B-LYMPHOCYTES. (lookformedical.com)
  • Scott and Rob-Smith have reported a neoplastic disorder, showing hemophagocytosing histiocytes and systemic proliferation of the precursors of histiocytes in 1939. (scirp.org)
  • Later came to be used to describe all forms of lymphoid neoplastic proliferations that arise as discrete tissue masses. (fdocuments.net)
  • 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)
  • Cases previously reported as malignant histiocytosis were shown to be lymphomas of T or B lineage, especially anaplastic large-cell lymphomas. (bmj.com)
  • A case of malignant histiocytosis is described, in which a patient presenting with symptoms suggestive of pneumonia suddenly deteriorated and died. (bmj.com)
  • This is the second reported case of malignant histiocytosis presenting with spontaneous splenic rupture. (bmj.com)
  • With malignant histiocytosis, a high mortality rate. (antibiotic-store.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)
  • Patients with infection-associated HLH usually have persistent unexplained fever, cytopenia, lymphadenopathy, and, frequently, hepatosplenomegaly and coagulopathy, causing diagnostic difficulties with malignant histiocytosis or T-cell lymphoma ( 2 ) . (cdc.gov)
  • Epstein-Barr virus (EBV) infection has been prominently associated with HPS, with clonal proliferation and the hyperactivation of EBV-infected T cells. (biomedcentral.com)
  • Nonmalignant Nonneoplastic - Reactive (cutaneous or systemic) histiocytosis. (petmoo.com)
  • Hemophagocytic lymphohistiocytosis (HLH) is an uncommon syndrome characterized by a reactive, systemic proliferation of benign histiocytes throughout the reticuloendothelial system ( 1 ) . (cdc.gov)
  • [13] Histologically, the cutaneous infiltrate includes a mixture of foamy histiocytes, lymphocytes, fibroblasts and multinucleated giant cells, including Touton-type giant cells, with a moderate amount of collagen deposition (Figures 4-5). (eyewiki.org)
  • Figure 5: High-power view reveals a mixture of multinucleated giant cells, including Touton-type giant cells, foamy histiocytes, lymphocytes, and fibroblasts with a moderate amount of collagen deposition. (eyewiki.org)
  • Histiocytosis can be both a systemic neoplasm, and can be expressed in the formation of benign single granulomas. (antibiotic-store.com)
  • Bone marrow aspirate showing phagocytosis of neutrophil, nucleated erythrocyte, and platelets by benign histiocytes (Wright stain, x400). (cdc.gov)
  • Sinus histiocytosis with massive lymphadenopathy: A newly recognized benign clinicopathological entity. (jbstjournal.com)
  • Juvenile xanthogranuloma (JXG) is a rare benign histiocytic proliferation that develops in infants and young children. (eyewiki.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)
  • 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)
  • Rosai-dorfman disease (RDD) or sinus histiocytosis with massive lymphadenopathy is a rare histiocytic proliferative disorder of unknown etiology first identified and characterized by Rosai and Dorfman in 1969. (jbstjournal.com)
  • This disorder is characterized by an abnormal increase in certain immune cells called histiocyte cells. (netlify.app)
  • Histiocytes are part of the reticuloendothelial system, on which the response of the child's immunity to a meeting with the infection depends. (antibiotic-store.com)
  • Hemophagocytic syndrome (HPS) is clinically defined as a combination of fever, liver dysfunction, coagulation abnormalities, pancytopenia, progressive macrophage proliferation throughout the reticuloendothelial system, and cytokine over-production, and may be primary or secondary to infectious, auto-immune, and tumoral diseases. (biomedcentral.com)
  • A number sign (#) is used with this entry because of evidence that histiocytosis and lymphadenopathy with or without cutaneous, cardiac, and/or endocrine features, joint contractures, and/or deafness (histiocytosis-lymphadenopathy plus syndrome) is caused by homozygous or compound heterozygous mutation in the SLC29A3 gene (612373) on chromosome 10q22. (findzebra.com)
  • The histiocytosis-lymphadenopathy plus syndrome comprises features of 4 histiocytic disorders previously thought to be distinct: Faisalabad histiocytosis (FHC), sinus histiocytosis with massive lymphadenopathy (SHML), H syndrome, and pigmented hypertrichosis with insulin-dependent diabetes mellitus syndrome (PHID). (findzebra.com)
  • FHC described an autosomal recessive disease involving joint deformities, sensorineural hearing loss, and subsequent development of generalized lymphadenopathy and swellings in the eyelids that contain histiocytes (summary by Morgan et al. (findzebra.com)
  • 2005) reported 3 Turkish brothers with sinus histiocytosis and massive lymphadenopathy (SHML). (findzebra.com)
  • Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): Report of a case and literature review. (jbstjournal.com)
  • 4. Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): Review of the entity. (jbstjournal.com)
  • In some cases, abnormal accumulation of histiocytes may occur in other areas of the body besides the lymph nodes (extranodal). (rarediseases.org)
  • In some cases of Rosai-Dorfman disease, the accumulation of histiocytes into masses may cause compression of vital organs potentially resulting in serious complications. (rarediseases.org)
  • 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)
  • This rare condition is characterized by the abnormal production and accumulation of immune system cells called histiocytes in many of the body's tissues. (medlineplus.gov)
  • The disease most commonly affects the bones, causing bone thickening and pain, but the accumulation of histiocytes can also cause signs and symptoms affecting the brain, eyes, lungs, liver, kidneys, and other organs. (medlineplus.gov)
  • 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)
  • 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)
  • 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 typical histologic appearance of JXG consists of a dense dermal infil- trate of foamy histiocytes, foreign body and Touton giant cells, the latter being nearly pathognomonic of JXG [ 1 ]. (who.int)
  • The lower left limb was shorter than filtrate of foamy histiocytes and eosi- tion, and haematological work-up was the right by 2 cm, with resultant limping. (who.int)
  • Hemophagocytic syndrome (HPS) is a potentially fatal condition due to dysregulated lymphocyte activation and proliferation, mainly characterised by impaired or inactive natural killer (NK) cells and cytotoxic T cells, which leads to macrophage hyperactivation and over-expression of cytokines [ 1 ]. (biomedcentral.com)
  • Histiocytic tumors are derived from the mononuclear-phagocytic and histiocyte system[ 1 - 3 ]. (oncotarget.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)
  • These diseases are related to other forms of abnormal proliferation of white blood cells, such as leukemias and lymphomas. (wikipedia.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)
  • The syndrome was first described in 1939 as poorly-controlled histiocyte proliferation, but has since also been called hemophagocytic histiocytosis and macrophage activation syndrome [ 5 - 7 ]. (biomedcentral.com)
  • Bone marrow aspirate revealed proliferation of histiocytes with prominent hemophagocytosis ( Figure ). (cdc.gov)
  • 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)
  • Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. (msdmanuals.com)
  • An allograft biopsy performed 6 months after the transplantation showed extensive glomerular infiltration of CD68 + foam cells (histiocytes) intermingled with many CD3 + T-cells (predominantly CD8 + cells). (biomedcentral.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)
  • Histologically, the biopsied lesion demonstrated the classic histiocyte proliferation which exhibited emperipolesis (engulfment of lymphocyte or plasma cells by large histiocyte) which are CD-68, S-100 positive, and CD-1a negative on immunohistochemistry. (jbstjournal.com)
  • citation needed] The disease has gone by several names, including Hand-Schüller-Christian disease, Abt-Letterer-Siwe disease, Hashimoto-Pritzker disease (a very rare self-limiting variant seen at birth) and histiocytosis X, until it was renamed in 1985 by the Histiocyte Society. (wikipedia.org)
  • Hand - Schuller - Christian disease is a variant of histiocytosis that is also rare. (antibiotic-store.com)
  • Specifically, the RAS/MAPK pathway regulates the growth and division (proliferation) of cells, the process by which cells mature to carry out specific functions (differentiation), cell movement (migration), and the self-destruction of cells (apoptosis). (medlineplus.gov)
  • Dendritic cells are a form of histiocyte, or white blood cell. (netlify.app)
  • Furthermore, frequent contact between glomerular T-cells and histiocytes, and the existence of activated CD8 + cells (CD8 + , HLA-DR + cells) were observed by double immunostaining. (biomedcentral.com)
  • The abnormal cells in LCH have abnormal proliferation and lower antigen-presenting capability. (capsulehealth.one)