Histiocytic disorders tumor dendritic cell histiocytosis. Medical search. Frequent questions

Single-system Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon type of Langerhans cell histiocytosis that affects only the lung. (histio.org)
Langerhans cell histiocytosis (LCH) is blood disease which is now recognized as a cancer and can affect virtually any organ system of the body. (histio.org)
The exact prevalence of single-system Pulmonary Langerhans cell histiocytosis (PLCH) is unknown. (histio.org)
A similar disease in humans, Hashimoto-Pritzker disease, is also a Langerhans cell histiocytosis. (wikipedia.org)
[ 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)
[ 3 , 4 ] 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)
[ 5 , 6 ] Although several histiocytic disorders are briefly discussed in this article (see History ), the primary focus is on Langerhans cell histiocytosis. (medscape.com)
Langerhans cell histiocytosis (LCH) can be localized and manifest as pain or may even be asymptomatic, as is the case in isolated bone lesions. (medscape.com)
We pursued target-capture next generation sequencing (NGS) on tissue specimen of patients with various histiocytic neoplasms- Erdheim-Chester disease (ECD), Langerhans cell histiocytosis (LCH), Rosai-Dorfman disease (RDD), histiocytic sarcoma (HS), and Langerhans cell sarcoma (LCS). (tempus.com)
Langerhans cell histiocytosis (LCH) is a proliferation of dendritic mononuclear cells with infiltration into organs locally or diffusely. (msdmanuals.com)
Pulmonary Langerhans Cell Histiocytosis Pulmonary Langerhans cell histiocytosis (PLCH) is proliferation of monoclonal Langerhans cells in lung interstitium and airspaces. (msdmanuals.com)
Langerhans cell histiocytosis (LCH) is a dendritic cell (antigen-presenting cell) disorder. (msdmanuals.com)
Symptoms and signs of Langerhans cell histiocytosis vary considerably depending on which organs are infiltrated. (msdmanuals.com)
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)
The pathogenesis of Langerhans cell histiocytosis (LCH) is unknown. (medscape.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)
Langerhans cell histiocytosis is an expression of myeloid dendritic cells, associated with a significant inflammatory component and varied systemic involvement. (bvsalud.org)
Erdheim-Chester disease (ECD) is a rare and probably fatal multisystemic non-Langerhans cell histiocytosis (LCH). (bvsalud.org)
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)

Histiocytoses encompass a group of diverse proliferative disorders characterized by the accumulation and infiltration of variable numbers of monocytes, macrophages, and dendritic cells in the affected tissues. (medscape.com)

Histiocytic neoplasms are rare disorders of the monocyte-macrophage-dendritic cell lineage that pose significant management challenges to the clinicians. (tempus.com)
In this study, we report the molecular alterations among patients with histiocytic neoplasms seen at our institution. (tempus.com)
We included 31 cases with histiocytic neoplasms in this study. (tempus.com)
Histiocytic neoplasms demonstrate diverse somatic oncogenic alterations. (tempus.com)
Myeloid neoplasms, predominantly MDS/AML, developing in patients, usually at a young age, with a familial platelet disorder and germline monoallelic RUNX1 mutations are categorized as myeloid neoplasms with germline RUNX1 mutation. (cornell.edu)

RUNX1 mutations have been reported in approximately 10% of myelodysplastic cases, 5-15% of acute myeloid leukemia, 8-37% of chronic myelomonocytic leukemia, 10% of T cell acute lymphoblastic leukemia, 3% of systemic mastocytosis, 2% of essential thrombocythemia and 2% of polycythemia vera. (cornell.edu)
RUNX1 mutations are independently associated with unfavorable outcomes and shorter survival after hematopoietic stem cell transplantation in patients with myelodysplastic syndrome and myelodysplastic syndrome/acute myeloid leukemia. (cornell.edu)

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)
Host recognition of microbes happens through pattern reputation receptors, including Toll-like receptors (TLRs)2 that are indicated on many cells, including macrophages, monocytes (1), and keratinocytes (2). (cancer-ecosystem.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)
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)
A group of heterogeneous lymphoid tumors generally expressing one or more B-cell antigens or representing malignant transformations of B-lymphocytes. (lookformedical.com)
Any of a group of malignant tumors of lymphoid tissue that differ from HODGKIN DISEASE, being more heterogeneous with respect to malignant cell lineage, clinical course, prognosis, and therapy. (lookformedical.com)
A group of heterogeneous lymphoid tumors representing malignant transformations of T-lymphocytes. (lookformedical.com)
Malignant lymphoma composed of large B lymphoid cells whose nuclear size can exceed normal macrophage nuclei, or more than twice the size of a normal lymphocyte. (lookformedical.com)
Malignant lymphoma in which the lymphomatous cells are clustered into identifiable nodules within the LYMPH NODES. (lookformedical.com)

Histiocytic disorders refer to diseases that are caused by abnormal behavior of these cells. (wikipedia.org)
Such a description excludes diseases in which infiltration of these cells occurs in response to a primary pathology. (medscape.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)

Rosai-Dorfman disease (RDD) is an uncommon, benign histiocytic proliferative disorder of unknown origin. (surgicalneurologyint.com)

[ 12 ] These cells can capture antigen and migrate to lymphoid organs, where they present the antigens to naive T cells. (medscape.com)
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)
Lymphoid cells concerned with humoral immunity. (lookformedical.com)
They acquire mucosa-associated lymphoid tissue (MALT) type as a result of an immunologically mediated disorder. (lookformedical.com)
B-cell lymphoid tumors that occur in association with AIDS. (lookformedical.com)

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 histiocyte is a differentiated tissue cell that has its origin in the bone marrow. (wikipedia.org)
As a result, too much tissue develops and tumors are formed. (newworldencyclopedia.org)
All platforms used formalin-fixed paraffin-embedded tumor tissue specimens for detection of substitutions, insertion and deletion alterations, and copy number alterations, and gene rearrangements. (tempus.com)
Cells grown in vitro from neoplastic tissue. (lookformedical.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)

Because these syndromes may be varied manifestations of the same underlying disorder and because most patients with LCH have manifestations of more than one of these syndromes, the designations of the separate syndromes (except for eosinophilic granuloma) are now mostly of historical significance. (msdmanuals.com)

Spontaneous regression is common in these tumors, and it is mediated by infiltration of CD8-expressing T cells followed by expression of Type 1 T helper cell cytokines (such as Interferon-gamma) and recruitment of antitumour effector cells. (wikipedia.org)
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)
Deregulated manifestation of the cytokines continues to be implicated in tumor, autoimmunity, and hyper-inflammatory areas (7C9). (cancer-ecosystem.com)

A histiocytoma in the dog is a benign tumor. (wikipedia.org)
Rosai Dorfman disease is a rare but benign and self-limiting disorder. (bvsalud.org)

[ 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)

In mammalian cells, the miRNAs give a key degree of natural rules in developmental and differentiation pathways (18). (cancer-ecosystem.com)

Each category of histiocytosis can be traced to reactive or neoplastic proliferation in one of these cell lineages. (medscape.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)

Improved understanding of the pathology of histiocytic disorders requires knowledge of the origins, biology, and physiology of the cells involved. (medscape.com)

DNA-microarray analysis of Burkitt's lymphoma and diffuse large B-cell lymphoma (DLBCL) showing differences in gene expression patterns. (newworldencyclopedia.org)
A form of non-Hodgkin lymphoma having a usually diffuse pattern with both small and medium lymphocytes and small cleaved cells. (lookformedical.com)

Over the last several decades, 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)
The clinical manifestations of histiocytosis depend on the organs and systems involved, as well as their level of involvement. (medscape.com)
The clinical significance of this finding in this tumor type is unknown. (cornell.edu)
Anti-FGFR2 agents are actively under multiple clinical trials against many types of solid tumor, including lung squamous cell carcinoma, gastric cancer, endometrial cancer, and cholangiocarcinoma. (cornell.edu)

In lymphoma, the cells in the lymphatic system grow abnormally, dividing too rapidly and growing without any order or control (Longe 2005). (newworldencyclopedia.org)
green indicates genes that are overexpressed in normal cells compared to lymphoma cells and red indicates genes that are overexpressed in lymphoma cells compared to normal cells. (newworldencyclopedia.org)
Lymphoma b cell. (lookformedical.com)
B-cell antigens are expressed on the immature cells that make up the tumor in virtually all cases of Burkitt lymphoma. (lookformedical.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)

RDD shows some variability in the involvement of the entire neuraxis, and because its ability to mimic meningeal and primary brain tumors, it is essential to be aware of this entity and consider RDD in the differential diagnosis of various lesions of the CNS. (surgicalneurologyint.com)
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)

Monoclonal antibodies, such as blinatumomab, may induce changes in the body's immune system and may interfere with the ability of cancer cells to grow and spread. (policylab.us)
Giving monoclonal antibody therapy with chemotherapy may kill more cancer cells. (policylab.us)

[ 1 ] only in recent years, with the application of molecular analyses, has the pathophysiology of these disorders begun to be elucidated. (medscape.com)

It is an abnormal growth in the skin of histiocytes (histiocytosis), a cell that is part of the immune system. (wikipedia.org)
Normal histiocytes originate from pluripotent stem cells, which can be found in bone marrow. (medscape.com)

Langerhans cells are dendritic cells found in the skin and function by internalizing antigens (foreign particles) and presenting them to T cells. (wikipedia.org)
The importance of dendritic cells in presenting antigens to T and B lymphocytes is increasingly recognized. (medscape.com)

As a result, the pathologic cells of LCH have been hypothesized to represent Langerhans cells in a state of arrested maturation. (medscape.com)
This mode of cell death serves as a balance to mitosis in regulating the size of animal tissues and in mediating pathologic processes associated with tumor growth. (lookformedical.com)

In LCH, abnormally proliferating dendritic cells infiltrate one or more organs. (msdmanuals.com)

One of the mechanisms by which CELL DEATH occurs (compare with NECROSIS and AUTOPHAGOCYTOSIS). (lookformedical.com)

In cell line and xenograft experiments, inhibition/knockdown of FGFR2 results in anti-tumour effects, suggesting the oncogenic role of FGFR2, raising the potential of FGFR2 as a target of therapy in FGFR2 driven cancers. (cornell.edu)
Both types of mutations have been shown to be potentially oncogenic in endometrial cancer cell lines. (cornell.edu)

Dendritic cells appear to develop in several pathways. (medscape.com)

Amplification of FGFR2 has been observed in lung adenocarcinoma, lung squamous cell carcinoma, endometrial carcinoma, urothelial carcinoma, germ cell tumor and breast cancers. (cornell.edu)

Ironically, the lymphatic system is fundamentally important for combating cancer cells-as well as foreign bodies, such as viruses and bacteria , and combating heart disease and arthritis as well. (newworldencyclopedia.org)
The only common feature among these tumors is the absence of giant REED-STERNBERG CELLS, a characteristic of Hodgkin's disease. (lookformedical.com)

Later, these were found to be manifestations of a single entity and were unified under the term histiocytosis X. (medscape.com)

Alterations in epigenetic regulators ( TET2, DMNT3A, KMT2A, ASXL1 ) were found in 5 (16%), potentially suggesting clonal hematopoiesis in a subgroup of histiocytosis. (tempus.com)

Pluripotent stem cells can also be committed to produce dendritic cells. (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)

The majority of mantle-cell lymphomas are associated with a t(11;14) translocation resulting in overexpression of the CYCLIN D1 gene (GENES, BCL-1). (lookformedical.com)

The information on this page has been written and reviewed by the Histiocytosis Association Board of Trustees Scientific Committee and a member of the Histiocyte Society , and subsequently audited by patients and families to ensure enough information was captured. (histio.org)
Histio UK also aims to support patients and their families as well as raise public and professional awareness of histiocytic disorders. (histiouk.org)

This classification attempts to classify lymphomas by cell type (i.e. the normal cell type that most closely resembles the tumor). (newworldencyclopedia.org)
A classification of B-lymphocytes based on structurally or functionally different populations of cells. (lookformedical.com)

The P253R variant in FGFR2 has also been described in some constitutional disorders including craniosynostosis syndromes (eg, Apert syndrome). (cornell.edu)

The tumors are often disseminated in unusual extranodal sites and chromosomal abnormalities are frequently present. (lookformedical.com)

B cells are lymphocytes (a class of white blood cells ) that play a large role in the adaptive immune system by making antibodies to identify and neutralize invading pathogens like bacteria and viruses . (newworldencyclopedia.org)
[ 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)
They are short-lived cells resembling bursa-derived lymphocytes of birds in their production of immunoglobulin upon appropriate stimulation. (lookformedical.com)

Previously thought to be an auto-immune disorder, LCH was classified as a blood cancer in 2008 by the World Health Organization. (histio.org)
Histiocytomas should never be treated with an intralesional injection of a corticosteroid, as remission relies on recognition of the tumour by the body's immune system which is suppressed by steroids. (wikipedia.org)
Specially, B cells play the major role in the humoral immune response , as opposed to the cell-mediated immune response that is governed by T cells , another type of lymphocyte. (newworldencyclopedia.org)
Lymphocyte-like natural killer (NK) cells also are involved in the immune system , albeit part of the innate immune system . (newworldencyclopedia.org)

The charity focuses on histiocytosis in babies, children, young people and adults. (histiouk.org)

These are distinguished by cell type (Longe 2005). (newworldencyclopedia.org)

Dog breeds that may be more at risk for this tumor include Bulldogs, American Pit Bull Terriers, American Staffordshire Terriers, Scottish Terriers, Greyhounds, Boxers, and Boston Terriers. (wikipedia.org)

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