CutaneousChildhood Langerhans Cell HistiocyPulmonaryDiagnosis of Langerhans cell histiocyClonalPLCHProliferationAbstractEosinophilicDendritic cell histiocytosisHistiocytesExtra Langerhans cellsTissuesUnifocalSymptomsLungDisorderSkeletalPaul LangerhansInvolvementEpidermalOrgansForm tumorsMacrophagesHistiocyticTumorsVulvaMonoclonalBone marrowMutationsBirbeckFindingsCD1aPathologicDiagnoseMultisystemMyeloidDiagnosticOccur at any ageAdultIncidenceAffectsCommonly
Cutaneous5
- When found in the skin it is called cutaneous single system Langerhans cell LCH. (wikipedia.org)
- 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 photoconjugation of psoralens with DNA produces an antiproliferative reaction in the skin, generates programmed cell death (apoptosis), and induces down-regulation of the cutaneous immune system. (medscape.com)
- A 2 yr old female intact flat-coated retriever dog was presented for evaluation of a histologically diagnosed cutaneous Langerhans cell histiocytosis of the muzzle with right mandibular nodal metastasis and suspected prescapular lymph node metastasis. (allenpress.com)
- Cutaneous histiocytosis was discovered in a 40-year-old man with a slow-growing nodule located on his right arm. (jamanetwork.com)
Childhood Langerhans Cell Histiocy1
- What Is Childhood Langerhans Cell Histiocytosis? (dana-farber.org)
Pulmonary15
- When found in the lungs, it should be distinguished from Pulmonary Langerhans cell hystiocytosis-a special category of disease most commonly seen in adult smokers. (wikipedia.org)
- Pulmonary Langerhans cell histiocytosis (PLCH) is a unique form of LCH in that it occurs almost exclusively in cigarette smokers. (wikipedia.org)
- Pulmonary Langerhans Cell Histiocytosis (PLCH) is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. (nih.gov)
- Single-system Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon type of Langerhans cell histiocytosis that affects only the lung. (histio.org)
- The exact prevalence of single-system Pulmonary Langerhans cell histiocytosis (PLCH) is unknown. (histio.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)
- Pulmonary Langerhans' cell histiocytosis (PLCH) remains an important diagnostic consideration in the differential diagnosis of diffuse infiltrative lung disease, particular among smokers. (elsevierpure.com)
- Vassallo, R & Limper, AH 2002, ' Pulmonary Langerhans' cell histiocytosis ', Seminars in Respiratory and Critical Care Medicine , vol. 23, no. 2, pp. 93-101. (elsevierpure.com)
- Pulmonary Langerhans cell histiocytosis is usually first diagnosed among adults. (wikidoc.org)
- Single-agent chemotherapy with cladribine (2-chlorodeoxyadenosine/2-CdA) may be a promising treatment for single-system pulmonary Langerhans cell histiocytosis. (medscape.com)
- Pulmonary Langerhans Cell Histiocytosis Pulmonary Langerhans cell histiocytosis (PLCH) is proliferation of monoclonal Langerhans cells in lung interstitium and airspaces. (msdmanuals.com)
- Effectiveness of cladribine therapy in patients with pulmonary Langerhans cell histiocytosis. (unil.ch)
- One example is pulmonary histiocytosis. (chkd.org)
- Single-system pulmonary langerhans cell histiocytosis with only tracheobronchial involvement: a case report. (bvsalud.org)
- Pulmonary Langerhans cell histiocytosis (PLCH) only with airway involvement manifested as diffuse thickening of the tracheobronchial walls is rare. (bvsalud.org)
Diagnosis of Langerhans cell histiocy4
- CT scan may be helpful in the diagnosis of Langerhans cell histiocytosis. (wikidoc.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)
Clonal6
- 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)
- 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 a clonal, pleo-morphic disease of unknown aetiology, with theaccumulation of local or disseminated atypical histiocyticcells staining positively for S-100 and CD-1a, and causingdamage in the bones, lungs, mucocutaneous structuresand endocrine organs.1 The condition is generally dia-gnosed in infancy and childhood, but onset in adulthoodcan occasionally occur. (uludag.edu.tr)
- 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)
PLCH3
- 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)
- It is hypothesized that bronchiolar destruction in PLCH is first attributed to the special state of Langerhans cells that induce cytotoxic T-cell responses, and this is further supported by research that has shown an abundance of T-cells in early PLCH lesions that are CD4+ and present early activation markers. (wikipedia.org)
- 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. (nih.gov)
Proliferation6
- 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)
- These diseases are related to other forms of abnormal proliferation of white blood cells, such as leukemias and lymphomas. (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)
- Langerhans cell histiocytosis (LCH) is a rare disease that involves the abnormal proliferation of Langerhans cells in the skin. (osmosis.org)
- The abnormal cells in LCH have abnormal proliferation and lower antigen-presenting capability. (capsulehealth.one)
- Langerhans cell histiocytosis (LCH) is a proliferation of dendritic mononuclear cells with infiltration into organs locally or diffusely. (msdmanuals.com)
Abstract1
- abstract = "IL-17A is a T cell-specific cytokine(1) that is involved in chronic inflammations, such as Mycobacterium infection(2), Crohn's disease(3), rheumatoid arthritis(4) and multiple sclerosis(5). (dtu.dk)
Eosinophilic5
- 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)
- Older names that were sometimes used for forms of Langerhans cell histiocytosis include eosinophilic granuloma, Hand-Schüller-Christian disease, and Letterer-Siwe disease. (medlineplus.gov)
- A surgical biopsy showed an infiltrate of eosinophilic cells with oval, grooved and convoluted nucleus, associated with eosinophils, lymphocytes and plasma cells ( Figure 2 ). (scirp.org)
- In immunohistochemical study, these eosinophilic cells expressed CD1a and S100 protein. (scirp.org)
- It consists of Langerhans cells with oval, grooved and convoluted nucleus and slightly eosinophilic cytoplasm, associated with eosinophils, lymphocytes and plasma cells (H & E × 400). (scirp.org)
Dendritic cell histiocytosis1
- 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)
Histiocytes7
- These cells in combination with lymphocytes, eosinophils, and normal histiocytes form typical LCH lesions that can be found in almost any organ. (wikipedia.org)
- The background shows reactive cellular infiltrates comprising eosinophils, neutrophils, histiocytes, lymphocytes and plasma cells. (radiologycases.my)
- 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)
- There are certain gene mutations that control how histiocytes function and cause Langerhans cell histiocytosis. (chkd.org)
- Langerhans cell histiocytosis (LCH) is a rare haematological neoplasm characterized by the accumulation of CD1a + , CD207/Langerin + histiocytes within inflammatory lesions. (biomedcentral.com)
- Outcomes From the French LCH registry [11], 9 individuals fulfilled the next inclusion requirements: i) new frozen biopsy cells and blood examples available, ii) raised percentage of lesions-infiltrating Compact disc207+ histiocytes ( 30%), iii) no mutation recognized by and with the i-plex mass spectrometric centered genotyping technology (Sequenom-Agena Bioscience) [12], iv) unfavorable testing for exon 2-3 mutations by Sanger sequencing. (careersfromscience.org)
- d Immunohistochemistry performed on FFPE examples from P5 demonstrated a solid cytoplasmic and nuclear positivity of histiocytes with phosphoERK1/2 (D13.14.4E, Rabbit mAb, Cell Signaling) in areas containing several Compact disc1a? (careersfromscience.org)
Extra Langerhans cells2
- In LCH, extra Langerhans cells spread through the blood and build up in certain parts of the body, where they can damage tissue or form tumors. (dana-farber.org)
- The extra Langerhans cells are a type of white blood cell. (chkd.org)
Tissues9
- citation needed] Multifocal multisystem LCH, also called Letterer-Siwe disease, is an often rapidly progressing disease in which Langerhans Cell cells proliferate in many tissues. (wikipedia.org)
- Other signs and symptoms that may occur in Langerhans cell histiocytosis, depending on which organs and tissues have Langerhans cell deposits, include swollen lymph nodes, abdominal pain, yellowing of the skin and whites of the eyes (jaundice), delayed puberty, protruding eyes, dizziness, irritability, and seizures. (medlineplus.gov)
- A PET/CT scan can provide information about both the structure and function of cells and tissues in the body during one imaging session. (dana-farber.org)
- They all share histologically a significant infiltration of affected tissues by langerhans cells. (scirp.org)
- X-rays and PET-CT scans may be performed to see if the affected tissues and cells are present. (homeremedylifestyle.com)
- We now know that epidermal Langerhans cells are not nerves but dendritic cells, a heterogeneous group of hematopoietic cells enriched in interface tissues and lymphoid organs. (capsulehealth.one)
- Langerhans cell histiocytosis causes damage to tissues all over the body. (chkd.org)
- Langerhans cell histiocytosis can cause damage to tissues and organs all over the body if it's not treated. (chkd.org)
- Langerhans cell histiocytosis is a rare disorder that damages tissues all over the body. (chkd.org)
Unifocal2
- Unifocal Langerhans cell histiocytosis and multifocal unisystem Langerhans cell histiocytosis are usually first diagnosed among individuals of 2-10 years of age. (wikidoc.org)
- Unifocal intranodal Langerhans' cell histiocytosis associated with metastatic breast carcinoma. (johnshopkins.edu)
Symptoms9
- The severity of Langerhans cell histiocytosis, and its signs and symptoms, vary widely among affected individuals. (medlineplus.gov)
- Langerhans cell histiocytosis (LCH) is a rare disease of unknown etiology, lacking an animal model, that cumulates symptoms that are found separately in various IL-17A-related diseases, such as aggressive chronic granuloma formation, bone resorption and soft tissue lesions with occasional neurodegeneration(11,12). (dtu.dk)
- 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 can cause symptoms in the skin, bones, lungs, and other parts of the body. (homeremedylifestyle.com)
- The symptoms of Langerhans' cell histiocytosis include pain, swelling, and brown or red skin sores. (homeremedylifestyle.com)
- Symptoms and signs of Langerhans cell histiocytosis vary considerably depending on which organs are infiltrated. (msdmanuals.com)
- Langerhans cell histiocytosis is a rare proliferative disorder of Langerhans cells with uncertain aetiology, wide spectrum of clinical symptoms and varied behaviour. (amedi.sk)
- What are the symptoms of Langerhans cell histiocytosis in a child? (chkd.org)
- Symptoms depend on where in the body the Langerhans cells build up. (chkd.org)
Lung3
- It is believed that cigarette smoke attracts specific immune cells in the lungs (Langerhans cells) which leads to a cascade of inflammation and injury to the air passages and lung tissue over time. (histio.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)
- Differential diagnoses included toxic lung damage or other interstitial lung disease, (e.g. atypical presentation of Langerhans cell histiocytosis or sarcoidosis). (cdc.gov)
Disorder7
- Langerhans cell histiocytosis is a disorder in which excess immune system cells called Langerhans cells build up in the body. (medlineplus.gov)
- In many people with Langerhans cell histiocytosis, the disorder eventually goes away with appropriate treatment. (medlineplus.gov)
- Langerhans cell histiocytosis is a rare disorder. (medlineplus.gov)
- Langerhans cell histiocytosis of the vulva is a rare disorder with few options for treatment. (nih.gov)
- Langerhans cell histiocytosis (LCH) is a rare disorder that occurs when a child has too many of a certain type of cell called Langerhans cells. (dana-farber.org)
- 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)
- Langerhans cell histiocytosis (LCH) is a dendritic cell (antigen-presenting cell) disorder. (msdmanuals.com)
Skeletal2
- 2013). "Skeletal involvement in Langerhans cell histiocytosis" . (wikidoc.org)
- Langerhans cell histiocytosis: skeletal sites commonly involved, age and incidence ratio F:M. The femoral region has been circled, showing the object of our case [1]. (pacs.de)
Paul Langerhans2
- 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)
Involvement2
- Langerhans cell hystiocitosis - Head and neck involvement. (pacs.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)
Epidermal8
- Although the epidermal Langerhans cell has been presumed to be the cell of origin in LCH, recent studies have called this belief into question. (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)
- 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)
- [ 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)
- At this point, Birbeck granules were thought to be exclusive to epidermal Langerhans cells, skin-restricted cells of the mononuclear phagocyte system. (capsulehealth.one)
- Histiocytosis X was renamed Langerhans-cell histiocytosis , reflecting the concept that LCH cells represented dysfunctional epidermal Langerhans cells. (capsulehealth.one)
- 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)
- 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)
Organs3
- On the other hand, the infiltration of organs by a monoclonal population of aberrant cells, the possibility of lethal evolution, and the cancer-based modalities of successful treatment are all consistent with a neoplastic process. (medscape.com)
- The treatment for Langerhans' cell histiocytosis will depend on the organs affected. (homeremedylifestyle.com)
- In LCH, abnormally proliferating dendritic cells infiltrate one or more organs. (msdmanuals.com)
Form tumors1
- In Langerhans cell histiocytosis, excess immature Langerhans cells usually form tumors called granulomas. (medlineplus.gov)
Macrophages4
- 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)
- In the 1970s , Steinman and Cohn distinguished dendritic cells from macrophages on the basis of specific morphologic features of dendritic cells and their superior capacity to present antigens to and activate antigen-specific T cells. (capsulehealth.one)
- LCH lesion also contains inflammatory cells and cytokines such as T lymphocytes, eosinophils, neutrophils, and macrophages. (capsulehealth.one)
- Immunologically, the cells presented an intermediate phenotype between Langerhans' cells and dermal macrophages. (jamanetwork.com)
Histiocytic1
- The tumour is composed of numerous histiocytic Langerhans cells displaying irregular nuclei with nuclear indentation and grooving, fine chromatin, inconspiciousto small nucleoli, and ample cytoplasm. (radiologycases.my)
Tumors1
- The cells build up and create tumors. (chkd.org)
Vulva2
- This report describes the histopathologic results of Langerhans cell histiocytosis of the vulva and options for treatment. (nih.gov)
- This summary addresses squamous cell cancer of the vulva and vulvar intraepithelial neoplasias (VIN), some of which are thought to be precursors to invasive squamous cell cancers. (cancer.gov)
Monoclonal1
- The antibody is a new monoclonal antibody against a C-type lectin found on the surface of Langerhans cells. (homeremedylifestyle.com)
Bone marrow2
- Langerhans cells are dendritic cells that arise from bone marrow and migrate to skin. (osmosis.org)
- Letterer-Siwe disease was described in infants with aggressive and generally fatal systemic disease, including skin, liver, spleen, and bone marrow infiltration by reticuloendothelial cells. (capsulehealth.one)
Mutations3
- Somatic mutations in the BRAF gene have been identified in the Langerhans cells of about half of individuals with Langerhans cell histiocytosis. (medlineplus.gov)
- Somatic gene mutations are acquired during a person's lifetime and are present only in certain cells. (medlineplus.gov)
- These neoplastic cells are the result of sporadic activating mutations in genes of the mitogen-activated protein kinase (MAPK) signaling pathway expressed by multipotent hematopoietic stem/progenitor cells or committed myeloid precursors [ 1 ]. (biomedcentral.com)
Birbeck3
- 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)
- 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)
Findings2
- Findings on CT scan suggestive of Langerhans cell histiocytosis include multiple osteolytic lesions causing full thickness bone destruction. (wikidoc.org)
- Immunohistochemical staining of the specimen was positive for S100+ CD1a+ and negative for CD43 and myeloperoxidase, findings that confirmed a diagnosis of skin-limited congenital Langerhans-cell histiocytosis. (medizzy.com)
CD1a1
- Immunohistochemistry: The Langerhans cells are positive for S100, CD68 and CD1a. (radiologycases.my)
Pathologic1
- As a result, the pathologic cells of LCH have been hypothesized to represent Langerhans cells in a state of arrested maturation. (medscape.com)
Diagnose2
- Another way to diagnose Langerhans cell histiocytosis is by examining the lymph nodes. (homeremedylifestyle.com)
- A healthcare provider may diagnose Langerhans cell histiocytosis through several things. (chkd.org)
Multisystem3
- Multisystem Langerhans' cell histiocytosis, however, is fatal in severe cases. (homeremedylifestyle.com)
- Multifocal multisystem Langerhans cell histiocytosis is usually first diagnosed among individuals younger than 2 years of age. (wikidoc.org)
- No consensus exists for the optimal therapy for Langerhans cell histiocytosis (LCH), particularly in the case of multisystem organ disease. (medscape.com)
Myeloid2
- Using a more sensitive technique for detection of mutant BRAF alleles (droplet digital PCR) [ 9 ], BRAF V600E was not detected in DNA extracted from myeloid or lymphoid cells isolated by flowcytometric sorting from a peripheral blood sample collected at the time of the fourth bone sample. (biomedcentral.com)
- Langerhans cell histiocytosis (LCH) can be an inflammatory myeloid neoplasia with constitutive activation from the MAPKinase RAS-RAF-MEK-ERK cell signaling pathway. (careersfromscience.org)
Diagnostic1
- Localized Langerhans cell histiocytosis (LCH) of bone often presents as a diagnostic challenge. (cmich.edu)
Occur at any age1
- Langerhans cell histiocytosis may occur at any age, but is most common in young children under 10. (dana-farber.org)
Adult1
- cell histiocytosis in the adult lumbar spine: case report. (pacs.de)
Incidence1
- The incidence of Langerhans cell histiocytosis is approximately 0.5 per 100,000 individuals in the United States. (wikidoc.org)
Affects1
- Langerhans cell histiocytosis usually affects Caucasian and Hispanic individuals. (wikidoc.org)
Commonly1
- Males are more commonly affected with Langerhans cell histiocytosis than females. (wikidoc.org)