Langerhans cell histiocytosis (LCH) is a rare disease involving clonal proliferation of It typically has no extraskeletal involvement, but rarely an identical lesion can be found in the skin, lungs, or stomach. When found in the lungs, it should be. Aug 28, Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder most in Erdheim-Chester disease but not in other non-Langerhans cell. Langerhans Cell Histiocytosis information from the Histiocytosis Association. To date, no large-scale studies have been done on how often LCH occurs in.

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Rare Disease Database

Pediatr Pathol Lab Med ; Up-front therapy for LCH: Neurodegenerative syndrome is treated with cytarabine or intravenous immunoglobulin. Central diabetes insipidus as presenting symptom of Langerhans cell histiocytosis.

The maturation stage of the dendritic cell will determine what type of disease a patient will develop. Investigational Therapies It is preferable that patients be treated on clinical trials so the biology and therapy of these rare hishiocitosis can be advanced. It is very important that children presenting with skin LCH have a complete evaluation to ensure there is histlocitosis other disease site. Children and adults may have LCH in skin macular, papular, ulcerative, or seborrheic rashesbones painful lytic lesionslymph nodes, brain pituitary, cerebrum and cerebellum lung, liver, spleen, and bone marrow.


Among children under the age of 10, yearly incidence is thought to be 1 in ,; [35] and in adults even rarer, in about 1 inInfiltration of the liver and spleen causes massive abnormal enlargement of organs organomegaly.

Clofarabine salvage therapy in refractory multifocal histiocytic disorders, including Langerhans cell histiocytosis, juvenile xanthogranuloma and Rosai-Dorfman disease. Subscribe to our Newsletter.


Cell-specific gene expression in Langerhans cell histiocytosis lesions reveals a distinct profile compared with epidermal Langerhans cells. Other complications include fractures of long bones and compression of vertebrae causing extreme pain and possibly spinal cord damage.

Patients may also have jaundice yellow color to the skin and the white part of the eyes. Symptoms of the following disorders can be similar to those of Langerhans cell histiocytosis. There are ongoing investigations to determine whether LCH is a reactive non-cancerous or neoplastic cancerous process.

It is preferable that patients be treated on clinical trials so the biology and therapy of these rare patients can be advanced. Recurrent cytogenetic or genomic abnormalities would also be required to demonstrate convincingly that LCH is a malignancy. The magazine, referring to the Spanish-speaking pediatric, indexed in major international databases: Oncology, Pizzo and Poplack eds. Robbins and Cotran pathologic basis of disease.

The latter may be evident in chest X-rays with micronodular and interstitial infiltrate in the mid and lower zone of lung, with sparing of the Costophrenic angle or honeycomb appearance in older lesions.


Orphanet Journal of Rare Diseases.

The pathogenesis of Langerhans cell histiocytosis LCH is a matter of debate. LCH usually affects children between 1 and 15 years old, with a peak incidence between 5 and 10 years of age. CiteScore measures average citations received per document published. Optimal therapy for adults with Langerhans cell histiocytosis bone lesions. Hispanics have a higher histioditosis of diffuse LCH involvement and Blacks are less affected.

European Journal of Cancer.

Coughing up blood hemoptysis is rare. LCH is usually a sporadic and non- hereditary condition but familial clustering has been noted in limited number of cases. Excellent for single-focus disease.

Langerhans cell histiocytosis – Wikipedia

Phillips CT of the skull if mastoids involved. International Journal of Pediatric Otorhinolaryngology. Histiocytic sarcoma Langerhans cell sarcoma Interdigitating dendritic cell sarcoma Follicular dendritic cell sarcoma. Nuclear and cytoplasmic staining S The specific underlying cause of ECD is unknown.

These patients may present with pus draining from the ears and thought to have an infection. MRI and CT may show infiltration in langeerhans turcica. British Journal of Haematology.

LCH provokes a non-specific inflammatory responsewhich includes fever, lethargyand weight loss. Clinically, its manifestations range from isolated bone lesions to multisystem disease.