Langerhans' cell histiocytosis

Langerhans' cell histiocytosis

Histiocytosis X, Langerhans cell granulomatosis, multifocal eosinophilic granuloma Hematology An autonomous proliferation of a lymphoreticular cell, the Langerhans cell, which stains positively with antibodies to ATPase, S-100 and CD1a; LC aggregates are accompanied by eosinophils, foamy cells, neutrophils, fibrosis; histiocytosis X is divided into 3 clinical forms
Langerhans cell histiocytosis–histiocytosis X  
Solitary bone involvement Eosinophilic granuloma A lesion of younger Pts; may affect any bone–spares hands & feet, most commonly, the cranial vault, jaw, humerus, rib & femur. Radiology Mimics Ewing sarcoma. Treatment Curettage. Prognosis Excellent
Multiple bone involvement Polyostotic eosinophilic granuloma, Hand-Schüller-Christian disease
A lesion that variably affects the skin, accompanied by proptosis, diabetes insipidus, or chronic otitis media or combination thereof, marked by a prolonged course with waxing and waning symptoms Prognosis Relatively good
Multiple organ involvement Letterer-Siwe disease A lesion that affects bone, lung and skin, which while histologically indistinct, but more aggressive than the other forms Prognosis Poor if < 18 months at time of diagnosis, hemorrhagic skin lesions, hepatomegaly, anemia, thrombocytopenia, BM involvement
References in periodicals archive ?
Langerhans' cell histiocytosis of the temporal bone in children.
An MRI revealed Isabella has a rare brain tumour called Langerhans' cell histiocytosis (LCH), which can stop the body's ability to retain liquid.
Rare DPLD with Lymphangioleiomyomatosis (LAM) well-defined features Langerhans' cell histiocytosis (LCH) 4.
Mr Bull, who also played for England, raised the money through two golf days and a celebrity race at Silverstone after hearing about 13-year-old cancer patient Jade Baugh from Wolverhampton, who has been a patient at the hospital since the age of one and is in remission from Langerhans' Cell Histiocytosis.
Andrew, 14, has Langerhans' Cell Histiocytosis (LCH).
Three months later, she was diagnosed with cancer-like disease Langerhans' cell histiocytosis (LCH) and diabetes insipidus - a serious kidney condition.
Langerhans' cell histiocytosis occurs as a consequence of accumulation and or proliferation of one of the cell types of histocytes.
A case of multiorgan Langerhans' cell histiocytosis presented with pneumothorax.
The differential diagnosis includes an eurysmal bone cysts, chondroblastoma, chondromyxoid fibroma, giant cell reparative granuloma, nonossifying fibroma, Langerhans' cell histiocytosis, synovial sarcoma, and high-grade central osteosarcoma.
Risk of secondary leukemia after treatment with etoposide (VP-16) for Langerhans' cell histiocytosis in Italian and Austrian-German populations.
Langerhans' cell histiocytosis of the temporal bone: Successful treatment of sensorineural hearing loss with low-dose radiotherapy.

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