• Langerhans cell histiocytosis is a disorder in which excess immune system cells called Langerhans cells build up in the body. (medlineplus.gov)
  • In Langerhans cell histiocytosis, excess immature Langerhans cells usually form tumors called granulomas. (medlineplus.gov)
  • Many researchers now consider Langerhans cell histiocytosis to be a form of cancer, but this classification remains controversial. (medlineplus.gov)
  • 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)
  • Langerhans cell histiocytosis is often diagnosed in childhood, usually between ages 2 and 3, but can appear at any age. (medlineplus.gov)
  • The severity of Langerhans cell histiocytosis, and its signs and symptoms, vary widely among affected individuals. (medlineplus.gov)
  • 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)
  • 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)
  • Somatic mutations in the BRAF gene have been identified in the Langerhans cells of about half of individuals with Langerhans cell histiocytosis. (medlineplus.gov)
  • Approximately 50% to 60% of patients with Langerhans cell histiocytosis (LCH) have the BRAF -V600E mutation. (cincinnatichildrens.org)
  • Pulmonary Langerhans Cell Histiocytosis (PLCH) is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. (nih.gov)
  • [ 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)
  • 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)
  • 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)
  • What Is Childhood Langerhans Cell Histiocytosis? (dana-farber.org)
  • 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)
  • Langerhans cell histiocytosis may occur at any age, but is most common in young children under 10. (dana-farber.org)
  • How is Langerhans cell histiocytosis diagnosed? (dana-farber.org)
  • What are Langerhans cell histiocytosis treatment options? (dana-farber.org)
  • Broadly speaking, they can be divided into Langerhans and non-Langerhans cell histiocytoses. (thedoctorsdoctor.com)
  • Langerhans cell histiocytoses are all defined by the presence of a unique ultrastructural organelle, the Birbeck granule. (thedoctorsdoctor.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)
  • The most common type is Langerhans cell histiocytosis, which occurs when the body accumulates too many immature Langerhans cells. (tgh.org)
  • 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)
  • CT scan may be helpful in the diagnosis of Langerhans cell histiocytosis. (wikidoc.org)
  • Findings on CT scan suggestive of Langerhans cell histiocytosis include multiple osteolytic lesions causing full thickness bone destruction. (wikidoc.org)
  • 2013). "Skeletal involvement in Langerhans cell histiocytosis" . (wikidoc.org)
  • Class IIa dermal dendritic cell histiocytosis is a reactive increase in the number of non-Langerhans' cell histiocytes. (dermnetnz.org)
  • 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)
  • 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 pathogenesis of Langerhans cell histiocytosis (LCH) is unknown. (medscape.com)
  • 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)
  • Clinical and microbiological evaluation of the oral cavity of patients with Langerhans Cell Histiocytosis. (authorea.com)
  • The Langerhans Cell Histiocytosis (LCH) is a disease characterized by the clonal proliferation of Langerhans cells. (authorea.com)
  • 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)
  • C group: Non-Langerhans Cell Histiocytosis (skin). (petmoo.com)
  • L group: Erdheim-Chester Disease, Langerhans Cell Histiocytosis, etc. (petmoo.com)
  • Langerhans cell histiocytosis (LCH) is a rare disease that involves the abnormal proliferation of Langerhans cells in the skin. (osmosis.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)
  • 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)
  • Langerhans' cell histiocytosis is a rare disease . (homeremedylifestyle.com)
  • Children with Langerhans' cell histiocytosis have a high risk of developing cancer. (homeremedylifestyle.com)
  • Single-system Langerhans' cell histiocytosis responds well to chemotherapy. (homeremedylifestyle.com)
  • Multisystem Langerhans' cell histiocytosis, however, is fatal in severe cases. (homeremedylifestyle.com)
  • The treatment for Langerhans' cell histiocytosis will depend on the organs affected. (homeremedylifestyle.com)
  • 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)
  • Another way to diagnose Langerhans cell histiocytosis is by examining the lymph nodes. (homeremedylifestyle.com)
  • The best way to treat Langerhans' cell histiocytosis is to have the disease treated by experts who understand the disease. (homeremedylifestyle.com)
  • Approximately one out of every 200,000 children develops Langerhans cell histiocytosis every year. (homeremedylifestyle.com)
  • Langerhans cell histiocytosis is a rare disease that tends to affect children and adolescents. (wikidoc.org)
  • The incidence of Langerhans cell histiocytosis is approximately 0.5 per 100,000 individuals in the United States. (wikidoc.org)
  • Males are more commonly affected with Langerhans cell histiocytosis than females. (wikidoc.org)
  • Langerhans cell histiocytosis usually affects Caucasian and Hispanic individuals. (wikidoc.org)
  • African American individuals are less likely to develop Langerhans cell histiocytosis. (wikidoc.org)
  • Pulmonary Langerhans cell histiocytosis is usually first diagnosed among adults. (wikidoc.org)
  • Unifocal Langerhans cell histiocytosis and multifocal unisystem Langerhans cell histiocytosis are usually first diagnosed among individuals of 2-10 years of age. (wikidoc.org)
  • Multifocal multisystem Langerhans cell histiocytosis is usually first diagnosed among individuals younger than 2 years of age. (wikidoc.org)
  • 2.0 2.1 2.2 2.3 2.4 Langerhans Cell Histiocytosis Treatment (PDQ®): Health Professional Version. (wikidoc.org)
  • 4.0 4.1 4.2 4.3 4.4 Langerhans cell histiocytosis. (wikidoc.org)
  • 5.0 5.1 5.2 5.3 5.4 Langerhans cell histiocytosis. (wikidoc.org)
  • 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)
  • Localized histiocytosis is known as eosinophilic granuloma, as well as Langerhans cell granuloma. (antibiotic-store.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)
  • Histiocytosis X was renamed Langerhans-cell histiocytosis , reflecting the concept that LCH cells represented dysfunctional epidermal Langerhans cells. (capsulehealth.one)
  • Available at: http://www.cancernetwork.com/oncology-journal/langerhans-cell-histiocytosis-emerging-insights-and-clinical-implications [Accessed 1 Jul. (capsulehealth.one)
  • 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 is a rare proliferative disorder of Langerhans cells with uncertain aetiology, wide spectrum of clinical symptoms and varied behaviour. (amedi.sk)
  • Effectiveness of cladribine therapy in patients with pulmonary Langerhans cell histiocytosis. (unil.ch)
  • 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)
  • Histiocytosis (and malignant histiocytosis) are both important in veterinary as well as human pathology. (wikipedia.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)
  • Malignant histiocytosis is a rare invasive proliferation of neoplastic histiocytes. (bmj.com)
  • Cases previously reported as malignant histiocytosis were shown to be lymphomas of T or B lineage, especially anaplastic large-cell lymphomas. (bmj.com)
  • A case of malignant histiocytosis is described, in which a patient presenting with symptoms suggestive of pneumonia suddenly deteriorated and died. (bmj.com)
  • This is the second reported case of malignant histiocytosis presenting with spontaneous splenic rupture. (bmj.com)
  • M group: Includes Malignant Histiocytosis. (petmoo.com)
  • Malignant histiocytic diseases include malignant histiocytosis (now it is called localized and disseminated histiocytic sarcoma) and cutaneous histiocytoma, which is a benign histiocytic tumor. (petmoo.com)
  • Treatment protocol for malignant histiocytosis is directed towards controlling the local tumor and tackling the concern for metastasis. (petmoo.com)
  • Malignant histiocytosis cannot be completely cured even with medical management. (petmoo.com)
  • Malignant Histiocytosis is a potentially life-threatening disease in dogs but the mortality rate of dogs is not documented. (petmoo.com)
  • Malignant histiocytosis prognosis for recovery is poor. (petmoo.com)
  • With malignant histiocytosis, a high mortality rate. (antibiotic-store.com)
  • There are many different types of histiocytosis. (petmoo.com)
  • Cutaneous histiocytoses are classified according to the type of histiocyte within the skin. (dermnetnz.org)
  • Nonmalignant Nonneoplastic - Reactive (cutaneous or systemic) histiocytosis. (petmoo.com)
  • A number sign (#) is used with this entry because of evidence that histiocytosis and lymphadenopathy with or without cutaneous, cardiac, and/or endocrine features, joint contractures, and/or deafness (histiocytosis-lymphadenopathy plus syndrome) is caused by homozygous or compound heterozygous mutation in the SLC29A3 gene (612373) on chromosome 10q22. (findzebra.com)
  • 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)
  • It is impossible to avoid skin lesions with a generalized form of histiocytosis X. Most often the skin is affected in children. (antibiotic-store.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)
  • The North American Consortium for Histiocytosis (NACHO) is a group of institutions that collaborate on scientific and clinical research for histiocytic diseases. (wikipedia.org)
  • NACHO - North American Consortium for Histiocytosis. (wikipedia.org)
  • What Are the Symptoms of Histiocytosis? (tgh.org)
  • According to the Histiocytosis Association, 1 in 200,000 children in the United States are born with histiocytosis each year. (wikipedia.org)
  • The Blue Ribbon Lapel Pin has been used to promote public awareness of Hystiocytosis or Histiocytosis which affects approximatley 1 � 200,000 children from ages 0 - 15. (pinscentral.ca)
  • The histiocytosis-lymphadenopathy plus syndrome comprises features of 4 histiocytic disorders previously thought to be distinct: Faisalabad histiocytosis (FHC), sinus histiocytosis with massive lymphadenopathy (SHML), H syndrome, and pigmented hypertrichosis with insulin-dependent diabetes mellitus syndrome (PHID). (findzebra.com)
  • 2005) reported 3 Turkish brothers with sinus histiocytosis and massive lymphadenopathy (SHML). (findzebra.com)
  • Occasionally and confusingly, the term histiocytosis is sometimes used to refer to individual diseases. (wikipedia.org)
  • Later, these were found to be manifestations of a single entity and were unified under the term histiocytosis X. (medscape.com)
  • Each category of histiocytosis can be traced to reactive or neoplastic proliferation in one of these cell lineages. (medscape.com)
  • What is the Histiocyte Society classification of histiocytosis syndromes? (wikipedia.org)
  • The clinical manifestations of histiocytosis depend on the organs and systems involved, as well as their level of involvement. (medscape.com)
  • A histiocytosis is a disease in which there are too many histiocyte cells in the skin and other organs. (dermnetnz.org)
  • Histiocytosis is an umbrella term for an emerging spectrum of "syndromes" or disorders that involve abnormal proliferation of specialized white blood cells (histiocytes). (petmoo.com)
  • In medicine, histiocytosis is an excessive number of histiocytes (tissue macrophages), and the term is also often used to refer to a group of rare diseases which share this sign as a characteristic. (wikipedia.org)
  • 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)
  • In general, Histiocytosis refers to an abnormally large amount of histiocytes. (petmoo.com)
  • Skeletal survey is useful in children Ultrasound of liver and spleen Blood tests: measure cell counts and inflammation Breathing tests Tissue biopsy and molecular testing to detect mutations There are competing systems for classifying histiocytoses. (wikipedia.org)
  • Histiocytosis in children is a group of diseases that are quite rare in medical practice, proceed in different ways, but are combined by proliferative processes in the monocytic-macrophage system. (antibiotic-store.com)
  • Based on these findings, Cincinnati Children's has started treating histiocytosis patients with oral medicines that block these gene mutations. (cincinnatichildrens.org)
  • Cincinnati Children's researchers use genomic profiling of biopsies from treatment-resistant histiocytoses to identify drugs to successfully treat patients. (cincinnatichildrens.org)
  • The histological (associated with the microscopic structure of tissues) manifestations of various clinical variants of histiocytosis in their course do not have particularly significant differences. (antibiotic-store.com)
  • Learn more about the Histiocytosis Program on the Dana-Farber Cancer Institute website. (childrenshospital.org)
  • The diagnostic process for histiocytosis typically begins with a physical examination, blood work and imaging studies. (tgh.org)
  • HAA also states that most of the people diagnosed with histiocytosis are children under the age of 10, although the disease can afflict adults. (wikipedia.org)
  • Histiocytosis is a rare disease, thus its diagnosis may be challenging. (wikipedia.org)
  • 714-724 X-type histiocytoses Non-X histiocytoses Lymphohistiocytosis is a similar immune system disease characterized by the inappropriate activation of natural killer cells, CD8+ cytotoxic T-cells, and macrophages, involving principally the liver, spleen and central nervous system and associated with severe lymphoid atrophy. (wikipedia.org)
  • Hand - Schuller - Christian disease is a variant of histiocytosis that is also rare. (antibiotic-store.com)
  • 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)
  • 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)
  • What Are the Treatment Options for Histiocytosis? (tgh.org)
  • Some forms of histiocytosis resolve without treatment, but others may have a fatal outcome. (dermnetnz.org)
  • Histiocytosis can be both a systemic neoplasm, and can be expressed in the formation of benign single granulomas. (antibiotic-store.com)
  • Get your cause-related items now for the focus on Histiocytosis Awareness happening during September. (supportstore.com)
  • cell histiocytosis in the adult lumbar spine: case report. (pacs.de)
  • It may be possible to tell which type of histiocytosis is present by the appearance of the skin lesion or rash , but a skin biopsy is usually necessary. (dermnetnz.org)
  • 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 modalities used may include: Chemotherapy Cladribine (also known as 2CDA or Leustatin) Etoposide Methotrexate 6-mercaptopurine Vinblastine Surgery Radiation therapy Patients and families can gain support and educational materials from the Histiocytosis Association. (wikipedia.org)
  • Depending on the extent of the condition, histiocytosis may be treated with chemotherapy and/or steroids. (tgh.org)