Disorder histiocytoses. Medical search. Frequent questions

The most common type is Langerhans cell histiocytosis, which occurs when the body accumulates too many immature Langerhans cells. (tgh.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)
Approximately 50% to 60% of patients with Langerhans cell histiocytosis (LCH) have the BRAF -V600E mutation. (cincinnatichildrens.org)
Pulmonary Langerhans cell histiocytosis (PLCH) is proliferation of monoclonal Langerhans cells in lung interstitium and airspaces. (msdmanuals.com)
Langerhans Cell Histiocytosis Langerhans cell histiocytosis (LCH) is a proliferation of dendritic mononuclear cells with infiltration into organs locally or diffusely. (msdmanuals.com)
Pulmonary Langerhans cell histiocytosis is a disease in which monoclonal CD1a-positive Langerhans cells (a type of histiocyte) infiltrate the bronchioles and alveolar interstitium, accompanied by lymphocytes, plasma cells, neutrophils, and eosinophils. (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)
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)
Langerhans cell histiocytosis (LCH) is a rare disorder. (chkd.org)
Langerhans cell histiocytosis causes damage to tissues all over the body. (chkd.org)
What causes Langerhans cell histiocytosis in a child? (chkd.org)
There are certain gene mutations that control how histiocytes function and cause Langerhans cell histiocytosis. (chkd.org)
What are the symptoms of Langerhans cell histiocytosis in a child? (chkd.org)
How is Langerhans cell histiocytosis diagnosed in a child? (chkd.org)
A healthcare provider may diagnose Langerhans cell histiocytosis through several things. (chkd.org)
How is Langerhans cell histiocytosis treated in a child? (chkd.org)
Some parents of children with Langerhans cell histiocytosis may choose watchful waiting. (chkd.org)
What are possible complications of Langerhans cell histiocytosis in a child? (chkd.org)
Langerhans cell histiocytosis can cause damage to tissues and organs all over the body if it's not treated. (chkd.org)
Can Langerhans cell histiocytosis be prevented in a child? (chkd.org)
Experts don't know what causes Langerhans cell histiocytosis. (chkd.org)
Langerhans cell histiocytosis is a rare disorder that damages tissues all over the body. (chkd.org)
This report describes the histopathologic results of Langerhans cell histiocytosis of the vulva and options for treatment. (nih.gov)
Langerhans cell histiocytosis of the female genital tract is rare. (nih.gov)
Langerhans cell histiocytosis of the vulva is a rare disorder with few options for treatment. (nih.gov)
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)
OBJECTIVE: The etiology and pathogenesis of Langerhans cell histiocytosis (LCH) remain poorly understood. (thedoctorsdoctor.com)
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)
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)
I am an MRC Clinician Scientist and Honorary Consultant Paediatric Oncologist, providing a clinical service for young people with lymphoma or Langerhans cell histiocytosis at the Great North Children's Hospital, Newcastle. (ncl.ac.uk)
I also work closely with UK and European colleagues (iBFM, EICNHL, ESID) to develop our understanding of development of lymphoid malignancy in children with inherited immune deficiency or DNA repair disorders and provide the paediatric clinical service for Langerhans cell histiocytosis and collaborate with Newcastle Human Dendritic Cell Laboratory ( http://www.hudendritic.org/ ) run by Professor Matt Collin. (ncl.ac.uk)
Natal molars, in particular, have been linked to Pfieffer syndrome (a genetic disorder) and Langerhans cell histiocytosis (a white blood cell disorder). (thebump.com)
Reticulohistiocytoses are a type of non- Langerhans cell histiocytosis . (dermnetnz.org)
Our doctors provide unparalleled expertise in the diagnosis and treatment of the major types of histiocytosis - Langerhans cell histiocytosis (LCH) and hemophagocytic lymphohistiocytosis (HLH) - and treat rare, related disorders, such as juvenile xanthogranuloma and Rosai-Dorfman disease. (childrenshospital.org)
Our expertise also includes treating non-malignant conditions such as Langerhans cell histiocytosis, histiocytic and hemophagocytic lymphohistiocytosis (HLH) disorders, fibromatosis and benign tumors that may require surgery and surveillance. (cookchildrens.org)
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)
Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD) are characterized by a high frequency of BRAFV600E mutations, which are responsive to treatment with BRAF inhibitors such as vemurafenib. (targetedonc.com)
Opsin 3 expression in human Langerhans cell histiocytosis and its mediation of ELD-1 cellular function. (bvsalud.org)
Langerhans cell histiocytosis (LCH) is a type of -histiocytic disorder characterized by aberrant function, differentiation or proliferation of mononuclear phagocyte system cells , however, the pathogenesis is not fully understood. (bvsalud.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)

Sinus histiocytosis with massive lymphadenopathy (SHML), also designated as Rosai-Dorfman disease (RDD), is a rare benign reactive lymphoproliferative disorder. (bmj.com)
It is defined by a characteristic histopathology with sinus histiocytosis and haemophagocytosis known as emperipolesis. (bmj.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)

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)
Virus-associated hemophagocytic syndrome (VAHS) is a fulminant disorder associated with systemic viral infection and is characterized pathologically by the proliferation of hemophagocytic histiocytes in the lymphoreticular tissues. (cdc.gov)

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)
Improved understanding of the pathology of histiocytic disorders requires knowledge of the origins, biology, and physiology of the cells involved. (medscape.com)
Recent studies have shown that histiocytic disorders share the same genetic mutations as some cancers. (cincinnatichildrens.org)
Persons should consult with a licensed medical professional for the diagnosis and treatment of all histiocytic disorders and other health concerns. (histio.org)
The Histiocytosis Association solely seeks to provide knowledge, understanding and awareness of histiocytic disorders. (histio.org)

PH occurs along with other conditions like blood disorders and metabolic disorders. (clevelandclinic.org)
Marshfield Children's pediatric cancer specialists treat childhood cancers and blood disorders. (marshfieldclinic.org)
Search clinical trials for adult and pediatric cancers and blood disorders offered through Dana-Farber and our clinical partners. (dana-farber.org)
The Hematologic Malignancy Center at Dana-Farber/Boston Children's Cancer and Blood Disorders Center is one of the top pediatric leukemia and lymphoma treatment centers in the world. (dana-farber.org)
Children and teens with acute lymphoblastic leukemia are treated by our experts at Dana-Farber/Boston Children's Cancer and Blood Disorders Center through the Childhood Leukemia Program . (dana-farber.org)
We are committed to the idea that children with cancer and blood disorders deserve to be evaluated and treated close to home, without having to travel from their family's support systems. (cinj.org)
Children with cancer or blood disorders are also seen for consultations at Cooperman Barnabas Medical Center in Livingston . (cinj.org)
The Dana-Farber/Boston Children's Cancer and Blood Disorders Center provides expert treatment for newly diagnosed or recurrent histiocytosis, which is a cancer-like condition and may involve treatment with chemotherapy . (childrenshospital.org)
Images of peripheral blood and/or bone marrow of blood disorders and normal hematopoiesis. (hematology.org)
Complete cases of common blood disorders (peripheral blood, bone marrow, and diagnostic studies). (hematology.org)

Histiocytosis-lymphadenopathy plus syndrome (also known as SLC29A3 spectrum disorder) is a group of conditions with overlapping signs and symptoms that affect many parts of the body. (medlineplus.gov)
This group of disorders includes H syndrome, pigmented hypertrichosis with insulin-dependent diabetes mellitus (PHID), Faisalabad histiocytosis, and familial Rosai-Dorfman disease (RDD). (medlineplus.gov)
This spectrum is known as histiocytosis-lymphadenopathy plus syndrome because the disorders that make up the spectrum can have additional signs and symptoms. (medlineplus.gov)
the other forms are not considered part of histiocytosis-lymphadenopathy plus syndrome. (medlineplus.gov)
Histiocytosis-lymphadenopathy plus syndrome is a rare disorder, affecting approximately 100 individuals worldwide. (medlineplus.gov)
Histiocytosis-lymphadenopathy plus syndrome is caused by variants (also known as mutations) in the SLC29A3 gene, which provides instructions for making a protein called equilibrative nucleoside transporter 3 (ENT3). (medlineplus.gov)
The SLC29A3 gene variants involved in histiocytosis-lymphadenopathy plus syndrome reduce or eliminate the activity of the ENT3 protein. (medlineplus.gov)
Sea-blue histiocytosis is a cutaneous condition that may occur as a familial inherited syndrome or as an acquired secondary or systemic infiltrative process. (wikipedia.org)
Among the newly added codes are codes that better define metabolic disorders such as metabolic syndrome, also known as insulin resistance syndrome, dysmetabolic syndrome, hypertriglyceridemic waist, obesity syndrome, and Syndrome X. (codapedia.com)
Child must be diagnosed with cancer or a cancer predisposition (Beckwith-Wiedemann syndrome, Histiocytosis, Li-Fraumeni syndrome, Myelodysplastic syndrome (MDS), Post-Transplant Lymphoproliferative Disorders (PTLD), etc. (alexslemonade.org)

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 Histiocytosis Association provides the www.histio.org website solely for the general information and awareness of the visitor. (histio.org)
By using www.histio.org, and all other websites affiliated with the Histiocytosis Association, visitors agree to the terms and conditions presented herein this agreement and our privacy policy . (histio.org)
Under no circumstances shall the Histiocytosis Association or any of its staff, volunteers, board members, contributors or third-party service providers be liable for direct, indirect, exemplary, consequential, incidental or special damages that result from the direct or indirect use, or inability to use www.histio.org or other affiliated or linked websites. (histio.org)
By clicking on such links, visitors are leaving the Histiocytosis Association website, www.histio.org, and visiting external third-party sites. (histio.org)

One example is pulmonary histiocytosis. (chkd.org)
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)

Our expertise includes rare childhood tumors, histiocytosis, bone/brain tumors and clotting conditions. (marshfieldclinic.org)
he said, “was the consistent difficulty in identifying the BRAFV600E mutation in tissue biopsies from histiocytic disorder patients due to (1) heterogeneity in the cells infiltrating the tumors of these patients, and (2) frequent involvement in areas of the body that are difficult to biopsy and/or genotype in patients with these disorders, including bones and brain. (targetedonc.com)

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

Overview of Interstitial Lung Disease Interstitial lung diseases are a heterogeneous group of disorders characterized by alveolar septal thickening, fibroblast proliferation, collagen deposition, and, if the process remains unchecked. (msdmanuals.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)
Each category of histiocytosis can be traced to reactive or neoplastic proliferation in one of these cell lineages. (medscape.com)

This article provides a rational approach to determining the etiology of the lymph node disorder, highlights various disorders to consider in treating a child with lymphadenopathy, and discusses various means of obtaining a tissue diagnosis when the cause of lymphadenopathy is uncertain. (medscape.com)

Faisalabad histiocytosis typically causes lymphadenopathy and swelling of the eyelids due to accumulation of histiocytes. (medlineplus.gov)
A family history of lymphadenopathy suggests an underlying immune disorder, which occasionally results in malignancy. (medscape.com)

Histiocytosis can be categorized based on the type of immune cells involved: macrophages, monocytes or dendritic cells. (tgh.org)

What Are the Symptoms of Histiocytosis? (tgh.org)
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)

Because histiocytosis often behaves similarly to cancer, it is usually classified and treated as such. (tgh.org)
Previously thought to be an auto-immune disorder, LCH was classified as a blood cancer in 2008 by the World Health Organization. (histio.org)
In the past, this disorder was thought to be a type of cancer or a condition similar to cancer. (chkd.org)
In addition to treating blood cancers, our Center also treats histiocytosis, a condition that shares some of the characteristics of cancer. (dana-farber.org)
If your child, adolescent, or young adult has been diagnosed with cancer or a blood disorder, our team of experts can help. (cinj.org)
Learn more about the Histiocytosis Program on the Dana-Farber Cancer Institute website. (childrenshospital.org)

This disorder is likely an autoimmune response. (chkd.org)
Interpretation of perinatal clinical pathology tests: amniotic fluid chemistry, Kleihauer-Betke test, maternal-fetal antibody testing, serology of infection and autoimmune disorders, blood gas interpretation. (case.edu)

A feature common to the disorders in this spectrum is histiocytosis, which is the overgrowth of immune system cells called histiocytes. (medlineplus.gov)

By day 28, the lesion in MWCNT-exposed mice progressed to fibrotic granulomas, whereas it remained as alveolar histiocytosis in C60F-exposed mice. (cdc.gov)

The clinical manifestations of histiocytosis depend on the organs and systems involved, as well as their level of involvement. (medscape.com)

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

If histiocytosis is suspected, a physician will usually order a tissue biopsy or a bone marrow aspiration to confirm the diagnosis. (tgh.org)

The diagnostic process for histiocytosis typically begins with a physical examination, blood work and imaging studies. (tgh.org)

Type A insulin resistance is a rare genetic disorder resulting in mutation of the INSR gene, which prevents proper development of insulin receptors. (codapedia.com)

We offer novel treatments and comprehensive care for all forms of this rare disorder. (childrensnational.org)
ECD is a rare non-Langerhans form of histiocytosis with multisystemic infiltration. (iasp-pain.org)
Asked how these findings might affect the treatment of patients with these rare disorders, Abdel-Wahab pointed to the promising efficacy of RAF inhibitors (ie, vemurafenib) in treating patients with BRAFV600E -mutant histiocytic disorder, as seen in results from an ongoing clinical trial (NCT01524978). (targetedonc.com)

Cincinnati Children's researchers use genomic profiling of biopsies from treatment-resistant histiocytoses to identify drugs to successfully treat patients. (cincinnatichildrens.org)

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

Histiocytosis is an umbrella term that refers to a relatively uncommon group of syndromes associated with the overproduction of certain infection-fighting white blood cells known as histiocytes. (tgh.org)
Histiocytosis is a group of conditions that involve too many white blood cells (histiocytes) in your child's blood. (childrensnational.org)
Group 5 PH due to other disorders. (clevelandclinic.org)

We also collaborate with care providers from all over the world who are also involved in the field of histiocyte disorders, allowing us access to national and international approaches to treatment. (childrensnational.org)

Depending on the extent of the condition, histiocytosis may be treated with chemotherapy and/or steroids. (tgh.org)

Only a medical professional familiar with your overall health can diagnose histiocytosis and establish or modify a treatment plan. (histio.org)

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

Thus, IL-17A and IL-17A-stimulated DCs represent targets that may have clinical value in the treatment of LCH and other IL-17A-related inflammatory disorders. (dtu.dk)

What Are the Treatment Options for Histiocytosis? (tgh.org)
One of the trials we are pursuing will evaluate a new, MEK gene inhibitor as treatment for all patients with histiocytosis as frontline treatment. (cincinnatichildrens.org)
We participate in research treatment protocols with select institutions around the country who are investigating the newest approaches using the latest scientific advances for histiocyte disorders. (childrensnational.org)

Diseases3

Psychiatry and Psychology14

Information Science1

Langerhans Cell Histiocy52

Encompass a group of diverse1

Sinus3

Hemophagocytic2

Histiocytic disorders5

Blood disorders10

Syndrome10

Malignant histiocytosis1

Histio.org4

Pulmonary2

Tumors2

Benign1

Proliferation3

Etiology1

Lymphadenopathy2

Dendritic cells1

Symptoms3

Cancer6

Autoimmune2

Called histiocytes1

Alveolar1

Organs1

Pathophysiology1

Bone1

Typically1

Mutation1

Rare3

Biopsies1

Manifestations1

Group3

Collaborate1

Condition1

Diagnose1

Predominantly affects1

Inflammatory disorders1

Treatment3