• These cells in combination with lymphocytes, eosinophils, and normal histiocytes form typical LCH lesions that can be found in almost any organ. (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)
  • 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)
  • Solitary or multiple benign cutaneous nodules comprised of immature and mature vascular structures intermingled with endothelial cells and a varied infiltrate of eosinophils, histiocytes, lymphocytes, and mast cells. (lookformedical.com)
  • Prevention Gamma irradiation of cellular blood components to stop the proliferation of transfused lymphocytes. (ehd.org)
  • A skin biopsy from her face revealed a subepidermal clear zone, numerous foamy histiocytes throughout the dermis, dense cellularity, and few perivascular lymphocytes. (cdc.gov)
  • In LCH, abnormally proliferating dendritic cells infiltrate one or more organs. (msdmanuals.com)
  • The pathognomonic characteristic of HPS is the activation of well-differentiated macrophages, phagocyting erythrocytes, leukocytes and platelets in bone marrow, lymph nodes, spleen, liver and other organs, which can infiltrate almost anybody district and may account for many of its systemic features [ 3 ]. (biomedcentral.com)
  • In addition to damage to lung tissue, respiration vessels infiltrate with histiocytes. (antibiotic-store.com)
  • Open up in another windowpane Fig.?4 Thickened pericardium with infiltrate of histiocytes with both eosinophilic and somewhat foamy cytoplasm. (bioskinrevive.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)
  • Epstein-Barr virus (EBV) infection has been prominently associated with HPS, with clonal proliferation and the hyperactivation of EBV-infected T cells. (biomedcentral.com)
  • Langerhans cell histiocytosis is a rare disease involving clonal proliferation of langerhans cells seen in children and young adults. (scirp.org)
  • 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)
  • 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)
  • 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)
  • In some cases, abnormal accumulation of histiocytes may occur in other areas of the body besides the lymph nodes (extranodal). (rarediseases.org)
  • Granulomatous lesions of bone tissue, internal organs, and lymph nodes appear. (antibiotic-store.com)
  • It predominantly affects the lymph nodes, but can also be found extranodal in different organs. (surgicalneurologyint.com)
  • Clinically, It predominantly affects the lymph nodes but can also be found extranodally in different organs and usually presents with other constitutional symptoms such as fever, malaise, weight loss, and raised inflammatory markers. (surgicalneurologyint.com)
  • Microscopic findings are multifocal necrosis with syncytia in multiple organs, commonly including liver, spleen, lymph nodes and other lymphoid tissue(5). Hallmark syncytia of MHV infection are characterized by large degenerating cells at the periphery of necrotic foci, containing dense basophilic nuclei and nuclear remnants. (askjpc.org)
  • The characteristic feature of HLH appears to be an overgrowth of histiocytes with subsequent histiocytic phagocytosis found in the blood cells of the lymph nodes, bone marrow, liver and spleen. (e-cep.org)
  • These diseases are related to other forms of abnormal proliferation of white blood cells, such as leukemias and lymphomas. (wikipedia.org)
  • 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)
  • 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)
  • 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 disorder is characterized by an abnormal increase in certain immune cells called histiocyte cells. (netlify.app)
  • 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)
  • Microscopic review of the tissue specimen revealed proliferation of histiocytes (left) with positive CD1a staining, a marker for Langerhans' cells (right). (reviewofophthalmology.com)
  • Langerhans cell histiocytosis (LCH) is a proliferation of dendritic mononuclear cells with infiltration into organs locally or diffusely. (msdmanuals.com)
  • Гістіоцитоз легеневих клітин Лангерганса Pulmonary Langerhans cell histiocytosis (PLCH) is proliferation of monoclonal Langerhans cells in lung interstitium and airspaces. (msdmanuals.com)
  • Symptoms and signs of Langerhans cell histiocytosis vary considerably depending on which organs are infiltrated. (msdmanuals.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)
  • 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)
  • 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)
  • 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)
  • Dendritic cells are a form of histiocyte, or white blood cell. (netlify.app)
  • Histopathologically, nephrogenic systemic fibrosis resembles scleromyxedema in that it manifests with a proliferation of dermal fibroblasts and dendritic cells, thickened collagen bundles, increased elastic fibers, and mucin deposition. (medscape.com)
  • This disease is characterized by angioproliferative lesions resembling those of Kaposi sarcoma in the skin, liver, spleen, bone, and other organs. (medscape.com)
  • 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)
  • The cells of the cancer originate in the bone marrow but make lots of destructive lesions in the bone, skin, brain, lungs and blood forming organs. (histiouk.org)
  • On one end, the clinical spectrum includes an acute, fulminant, disseminated disease called Letterer-Siwe disease, and, on the other end, solitary or few, indolent and chronic lesions of bone or other organs called eosinophilic granulomas . (medscape.com)
  • Both solid organs and bone marrow Bone marrow The soft tissue filling the cavities of bones. (lecturio.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)
  • 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)
  • Later came to be used to describe all forms of lymphoid neoplastic proliferations that arise as discrete tissue masses. (fdocuments.net)
  • Scott and Rob-Smith have reported a neoplastic disorder, showing hemophagocytosing histiocytes and systemic proliferation of the precursors of histiocytes in 1939. (scirp.org)
  • Malignant tumors can invade surrounding tissue and spread to distant organs. (merckvetmanual.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)
  • Transplantation is a procedure that involves the removal of an organ or living tissue and placing it into a different part of the body or into a different person. (lecturio.com)
  • An increase in the number of cells in a tissue or organ without tumor formation. (lookformedical.com)
  • EBVaGC is defined by monoclonal proliferation of carcinoma cells with latent EBV infection, as demonstrated by EBV-encoded small RNA (EBER) in situ hybridization. (spandidos-publications.com)
  • 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)
  • 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)
  • The first reported case of hemophagocytic lymphohistiocytosis (HLH) was described in 1952 by Farquhar and Claireaux, [ 1 ] who called the disease familial hemophagocytic reticulosis and described it as a rare familial disorder characterized by a proliferation of histiocytes in solid organs and phagocytosis of blood cells. (medscape.com)
  • Nephrogenic systemic fibrosis (NSF), also known as nephrogenic fibrosing dermopathy (NFD), is a disease of fibrosis of the skin and internal organs reminiscent but distinct from scleroderma or scleromyxedema. (medscape.com)
  • latent infection can spread by b cell recirculation and proliferation, but whether this alone achieves systemic infection is unclear. (liverpool.ac.uk)
  • 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)
  • 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)
  • They confer resistance against many different viruses, inhibit proliferation of normal and malignant cells, impede multiplication of intracellular parasites, enhance macrophage and granulocyte phagocytosis, augment natural killer cell activity, and show several other immunomodulatory functions. (lookformedical.com)
  • Organs may be affected by infiltration, causing dysfunction, or by compression from adjacent enlarged structures. (msdmanuals.com)
  • The result of this process is uncontrolled and ineffective immune activation, multi-organ dysfunction, and hemophagocytosis throughout the reticuloendothelial system [ 2 ]. (biomedcentral.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)
  • The disease affects the internal organs, as well as the bones of the skeleton. (antibiotic-store.com)
  • Treatment for ECD is normally reserved for all those with symptomatic disease, asymptomatic CNS participation, or proof organ dysfunction. (bioskinrevive.com)
  • Owl's eye bodies - These are usually associated with the owl's eye inclusion bodies found in tissues infected with the cytomegalovirus infection, a disease which can cause multiple organ dysfunction. (pathologicalbodies.com)
  • Severe falciparum malaria is characterized by a number of very important organ dysfunction brought on primarily by the intensive sequestration of parasitized red blood cells in the microvasculature, together with the myocardial capillaries. (ehd.org)
  • In: Target Organ Pathology (Turton J, Hooson J, eds). (nih.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)
  • It can be seen as a cytokine storm disorder, representing a variety of inflammatory etiologies with the final common result of overwhelming inflammation, hemodynamic instability, multiple organ failure, and potentially death [ 4 ]. (biomedcentral.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)
  • Same as with "The Dungeon" and Single-celled organisms are organized using specialized cell organelles, while several cells may organize into larger structures, such as tissues and organ Single-celled organisms are organized using specialized cell organelles, while sever B cells are immune cells that provide protection against specific pathogens and disease through the production of antibodies. (netlify.app)
  • 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)
  • Increase in constituent cells in the PROSTATE, leading to enlargement of the organ (hypertrophy) and adverse impact on the lower urinary tract function. (lookformedical.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)
  • The altered signaling interferes with the normal development of many organs and tissues, resulting in the characteristic features of cardiofaciocutaneous syndrome. (medlineplus.gov)
  • It can appear as a single lesion in an organ, up to a large quantity of lesions in one organ. (wikipedia.org)
  • When multiple lesions are scattered throughout an organ, it can be called a multifocal unisystem variety. (wikipedia.org)
  • Hemophagocytic lymphohistiocytosis (HLH) is a frequently fatal and likely underdiagnosed disease involving a final common pathway of hypercytokinemia, which can result in end-organ damage and death. (medscape.com)
  • Overview of Vasculitis Vasculitis is inflammation of blood vessels, often with ischemia, necrosis, and organ inflammation. (merckmanuals.com)
  • Less often, some cases may affect various organ systems of the body and may potentially cause serious complications. (rarediseases.org)
  • Typically, the upper and lower respiratory tract and the kidneys are affected, but any organ may be. (merckmanuals.com)
  • Organ involvement can also cause more specific symptoms. (wikipedia.org)
  • Symptoms vary depending on the organs and systems affected. (merckmanuals.com)
  • In about half of patients, more than one organ is involved. (msdmanuals.com)