Disorders characterized by the accumulation of cells histiocyte disorders are langerhans. Medical search. Frequent questions

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)
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)
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)
This mutation leads to production of a BRAF protein that is abnormally active, which disrupts regulation of cell proliferation. (medlineplus.gov)
The unregulated cell proliferation of early melanocytes leads to a large patch of darkly pigmented skin characteristic of giant congenital melanocytic nevus. (medlineplus.gov)
LCH and all its subtypes are characterized by monoclonal migration and proliferation of specific dendritic cells. (wikipedia.org)
Regardless of the subtype of Langerhans cell histiocytosis, the pathologic hallmark for all subtypes of LCH is the abnormal proliferation and accumulation of immature Langerhans cells, macrophages, lymphocytes, and eosinophils. (wikipedia.org)
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)
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)
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)
The abnormal cells in LCH have abnormal proliferation and lower antigen-presenting capability. (capsulehealth.one)
Each category of histiocytosis can be traced to reactive or neoplastic proliferation in one of these cell lineages. (medscape.com)
Overview of Histiocytic Disorders The histiocytic disorders are clinically heterogeneous disorders that result from an abnormal proliferation of histiocytes that are either Dendritic cells (antigen-presenting cells) Monocyte-macrophages. (msdmanuals.com)
Histopathologically, VX is characterized by papillary or verrucous projections associated with the proliferation of pavimentous stratified epithelium with hyperparakeratosis and numerous foamy cells (histiocytes) 1 . (bvsalud.org)

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)
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 histiocytoses encompass a group of diverse disorders characterized by the accumulation and infiltration of variable numbers of monocytes, macrophages, and dendritic cells in the affected tissues. (medscape.com)
The histopathological aspect is characterized by the presence of macrophages with foam cytoplasm (xanthoma cells) confined to the soft papillary tissue. (bvsalud.org)
12 demonstrated that VX has macrophages containing lipids within them, anD after immunohistochemical studies, they confirmed the macrophagic origin of the xanthoma cells 13-14 , in which their cytoplasm is PAS-positive 2 . (bvsalud.org)

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)
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)
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)
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)

A type of granuloma characterized morphologically by the predominance of Langerhans cells with characteristic grooved, folded, indented nuclei in the appropriate milieu that includes variable numbers of eosinophils and histiocytes including multinucleated forms, often appearing similar to osteoclasts or touton like giant cells, neutrophils and small lymphocytes. (nih.gov)

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)
In 1868, Paul Langerhans discovered the epidermal dendritic cells that now bear his name. (medscape.com)

reticulum cell sarcomas, cutaneous nodular amyloidosis) are relatively common cutaneous tumors. (merckvetmanual.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)
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)
It is constitutively expressed in the skin and may play a role in T-CELL trafficking during cutaneous INFLAMMATION. (lookformedical.com)

Some of these uncommon lesions are not well characterized, with a variety of confusing nomenclature and classification schemes based upon historical precedent or an incomplete knowledge base. (medscape.com)
As understanding of the underlying pathophysiology and molecular biology of these lesions advances, the ability to classify, characterize, and treat them will hopefully also improve. (medscape.com)
The collection of these cells is what forms granulomatous lesions. (wikipedia.org)
The intermediate clinical form called Hand-Schüller-Christian disease is characterized by multifocal, chronic involvement and classically presents as the triad of diabetes insipidus, proptosis, and lytic bone lesions. (medscape.com)
Arguments supporting the reactive nature of LCH include the occurrence of spontaneous remissions, the extensive elaboration of multiple cytokines by dendritic cells and T-cells (the so-called cytokine storm) in LCH lesions, and the good survival rate in patients without organ dysfunction. (medscape.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)
They may further differentiate within chronic inflammatory lesions to EPITHELIOID CELLS or may fuse to form FOREIGN BODY GIANT CELLS or LANGHANS GIANT CELLS. (lookformedical.com)

1 It is associated with tissue infiltration by foamy histiocytes. (bmj.com)
In 1994, after three years of progressive neurological dysfunction, diagnosis of Erdheim-Chester disease was made by analysis of biopsies of the femur bones, showing infiltration with foamy histiocytes lacking Birbeck granules and S-100 protein, and with few lymphocytes. (bmj.com)
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)

The pathogenesis of Langerhans cell histiocytosis (LCH) is unknown. (medscape.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)
Epidemiologic study of Langerhans cell histiocytosis in children. (thedoctorsdoctor.com)

The importance of dendritic cells in presenting antigens to T and B lymphocytes is increasingly recognized. (medscape.com)
[ 13 ] Dendritic cells are also efficient stimulators of B-cell lymphocytes. (medscape.com)
Effective induction of antigen-specific T-cell responses requires interaction between the dendritic cells and T lymphocytes to prime the latter cells for expansion and subsequent immune responses. (medscape.com)
The first signal may involve interaction between an MHC I-bound and/or MHC II-bound peptide on an APC with the T-cell receptor (TCRs) on the effector lymphocytes. (medscape.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)
As a result, the pathologic cells of LCH have been hypothesized to represent Langerhans cells in a state of arrested maturation. (medscape.com)
Over the past 50 years, the nomenclature used to describe histiocytic disorders has substantially changed to reflect the wide range of clinical manifestations and the variable clinical severities of some disorders that have the same pathologic findings. (medscape.com)

Localized histiocytosis is known as eosinophilic granuloma, as well as Langerhans cell granuloma. (antibiotic-store.com)
In the mid-1900s, Farber and Lichtenstein noted that biopsy specimens from cases of fatal Letterer-Siwe disease and specimens from cases of clinically mild eosinophilic granuloma were indistinguishable, and the two pathologists hypothesized that these conditions represent manifestations of a common disorder. (capsulehealth.one)
[ 2 ] For example, the entity now referred to as Langerhans cell histiocytosis (LCH) was initially divided into eosinophilic granuloma, Hand-Schüller-Christian disease, and Abt-Letterer-Siwe disease, depending on the sites and severity. (medscape.com)

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 is a rare disorder characterized by an abnormal accumulation of histiocytes and massive lymphadenopathy, particularly in the neck and head. (msdmanuals.com)

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 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)

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)

Pathology then uses immunohistochemical characteristics, such as cell surface markers CD1a, CD207, and S-100, to help identify the Langerhans cells. (wikipedia.org)

Rosai-Dorfman disease (RDD) is an uncommon, benign histiocytic proliferative disorder of unknown origin. (surgicalneurologyint.com)
benign, self-healing, non-Langerhans was referred to the dermatology clinic No dysmorphic features were noted. (who.int)
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)

[ 5 , 6 ] Although several histiocytic disorders are briefly discussed in this article (see History ),the primary focus is on Langerhans cell histiocytosis. (medscape.com)
Improved understanding of the pathology of histiocytic disorders requires knowledge of the origins, biology, and physiology of the cells involved. (medscape.com)

Specialized phagocytic cells of the MONONUCLEAR PHAGOCYTE SYSTEM found on the luminal surface of the hepatic sinusoids. (lookformedical.com)
Mononuclear cells with pronounced phagocytic ability that are distributed extensively in lymphoid and other organs. (lookformedical.com)
The term mononuclear phagocyte system has replaced the former reticuloendothelial system, which also included less active phagocytic cells such as fibroblasts and endothelial cells. (lookformedical.com)

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)
Such a description excludes diseases in which infiltration of these cells occurs in response to a primary pathology. (medscape.com)

The relatively long-lived phagocytic cell of mammalian tissues that are derived from blood MONOCYTES. (lookformedical.com)

In some cases, abnormal accumulation of histiocytes may occur in other areas of the body besides the lymph nodes (extranodal). (rarediseases.org)
Dendritic cells display a large amount of MHC-peptide complexes at their surface and can increase the expression of costimulatory receptors and migrate to the lymph nodes, spleen, and other lymphoid tissues, where they activate specific T cells. (medscape.com)

[ 11 ] Immature dendritic cells respond to GM-CSF (not to macrophage colony-stimulating factor [M-CSF]) and become committed to generating dendritic cells, which are "professional" antigen-presenting cells (APCs). (medscape.com)

In addition to damage to lung tissue, respiration vessels infiltrate with histiocytes. (antibiotic-store.com)
FAC is characterized by an inflammatory infiltrate consisting of intraepithelial neutrophils and/or neutrophils invading the lumen of the criptae, with no other microscopic alteration of the colonic mucosa and, in particular, without the presence of signs of chronic inflammation. (nih.gov)

Broadly speaking, they can be divided into Langerhans and non-Langerhans cell histiocytoses. (thedoctorsdoctor.com)

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)

Characteristics of the cells may not be sufficient in many instances to yield a definitive diagnosis or indicate the probable behavior of the lesion. (merckvetmanual.com)

A form of hepatic steatosis characterized by the presence of large, lipid-laden vesicles in the affected hepatocytes. (nih.gov)

Letterer-Siwe disease, (LSD) or Abt-Letterer-Siwe disease, is one of the four recognized clinical syndromes of Langerhans cell histiocytosis (LCH) and is the most severe form, involving multiple organ systems such as the skin, bone marrow, spleen, liver, and lung. (wikipedia.org)
Available at: http://www.cancernetwork.com/oncology-journal/langerhans-cell-histiocytosis-emerging-insights-and-clinical-implications [Accessed 1 Jul. (capsulehealth.one)
IPF is now recognized as a distinct clinical disorder. (atsjournals.org)

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)
It includes the humoral immune response and the cell-mediated response and consists of a complex of interrelated cellular, molecular, and genetic components. (lecturio.com)

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)

Bone marrow is made up of a framework of connective tissue containing branching fibers with the frame being filled with marrow cells. (lecturio.com)
Transplanted tissue may be cells (e.g., hematopoietic stem cells Hematopoietic stem cells Progenitor cells from which all blood cells derived. (lecturio.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 symptoms and physical findings associated with Rosai-Dorfman disease vary greatly from one person to another depending upon the extent of the disorder and the specific organ systems affected. (rarediseases.org)
Many individuals with Rosai-Dorfman disease do not develop any additional symptoms of the disorder (asymptomatic). (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)
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)
Most recently, this designation was changed to Langerhans cell histiocytosis based on the suggestion by Nezelof that the Langerhans cell represented the primary cell involved in the pathophysiology of the disease. (medscape.com)
Erdheim-Chester disease is a rare, non-Langerhans form of systemic histiocytosis of unknown aetiology. (bmj.com)
handle the Virgil\'s in a disease, disorder and ask ' annotation. (lakesinclair.org)

It has necrotizing activity against tumor cell lines and increases ability to reject tumor transplants. (lookformedical.com)

Histiocytes, a type of white blood cell, are part of the body's immune system. (wikipedia.org)
Tolerance Tolerance Pharmacokinetics and Pharmacodynamics of the transplanted organ by the immune system Immune system The body's defense mechanism against foreign organisms or substances and deviant native cells. (lecturio.com)

and a variety of other immune cells. (lookformedical.com)
A monocyte chemoattractant protein that has activity towards a broad variety of immune cell types. (lookformedical.com)

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)

The disorder predominantly affects children, adolescents or young adults. (rarediseases.org)

They are characterised by bilateral, symmetric sclerosis of the metaphyseal regions of the long bones. (bmj.com)
Yellow marrow is found in the large cavities of large bones and consists mostly of fat cells and a few primitive blood cells. (lecturio.com)

Cell surface glycoproteins that bind to chemokines and thus mediate the migration of pro-inflammatory molecules. (lookformedical.com)

Many acute and chronic lung disorders with variable degrees of pulmonary inflammation and fibrosis are collectively referred to as interstitial lung diseases (ILDs) or diffuse parenchymal lung diseases. (atsjournals.org)

Focal active colitis (FAC) is characterized by focal crypt damage caused by neutrophils. (nih.gov)

The pathogenic mechanisms reported in the literature for VX comprise inflammation, virus, local trauma, lipid metabolism disorders and immunological changes 7,12,15 . (bvsalud.org)

Normal histiocytes originate from pluripotent stem cells, which can be found in bone marrow. (medscape.com)

from The Dictionary of Cell Biology, Lackie and Dow, 3rd ed. (lookformedical.com)

At least 49 BRAF mutations have been identified in people with this disorder. (medlineplus.gov)
Additional gene mutations in cells within the nevus after birth can lead to melanoma in people with giant congenital melanocytic nevus. (medlineplus.gov)

[ 12 ] These cells can capture antigen and migrate to lymphoid organs, where they present the antigens to naive T cells. (medscape.com)

This condition is characterized by a large, noncancerous patch of abnormally dark skin that is present from birth and an increased risk of a type of skin cell cancer called melanoma (described below). (medlineplus.gov)

This condition is characterized by multiple brown skin spots (lentigines), heart defects, short stature, a sunken or protruding chest, and distinctive facial features. (medlineplus.gov)
Immunodeficiency-40 is an autosomal recessive primary form of combined immunodeficiency mainly affecting T-cell number and function, with other more variable defects in B-cell and NK-cell function. (nih.gov)
almost, in defects with Upper tuberculosis, remission discusses here characterized in the example as a therapy of a child in the rare cramps. (lakesinclair.org)

Committed stem cells can mature to become antigen-processing cells, with some possessing phagocytic capabilities. (medscape.com)
and foreign particles by phagocytic cells (PHAGOCYTES). (lookformedical.com)

Histopathology of peripheral nerve and sympathetic ganglion from a patient with autonomic failure, oat-cell carcinoma of the lung, and positive anti-HU antibody titer. (medscape.com)

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