Langerhans' cell histiocytosis

Langerhans’ cell histiocytosis

A group of conditions characterised by proliferation of Langerhans cells, which are lymphoreticular cells. Langerhans cell aggregates are nodular, especially in the lungs, and are variably accompanied by eosinophils, foamy cells, neutrophils, and fibrosis.

Prognosis
Good if limited to a single system. Multi-system involvement carries a 10–20% mortality rate due to organ failure; 50–60% have chronic disease; 30–40% have long-term clinical remission.

Langerhans cell histiocytosis types 
Pulmonary LCH
A lesion virtually exclusive to cigarette smokers, which is a form of smoking-related interstitial lung disease. Smoking cessation may lead to reversal of changes or evolution to pulmonary fibrosis and pulmonary hypertension.
 
Unifocal LCH
Eosinophilic granuloma, solitary bone involvement
A lesion affecting younger patients; may affect any bone, most commonly the cranial vault, jaw, humerus, rib and femur (often spares the hands and feet).

Imaging
Mimics Ewing sarcoma.
 
Treatment
Curettage.
 
Prognosis
Excellent.
 
Multifocal unisystem
LCH Hand-Schüller-Christian disease, multiple bone involvement.

Polyostotic eosinophilic granuloma 
A lesion that may affect the skin, accompanied by proptosis, diabetes insipidus, or chronic otitis media or combination thereof, marked by a chronic course with waxing and waning symptoms.

Prognosis
Relatively good.
 
Multifocal multisystem LCH
Letterer-Siwe disease, multiple organ involvement
A lesion that affects bone, lung and skin; while histologically indistinct, it is more aggressive than the other forms.

Prognosis
Poor if < 18 months at time of diagnosis; haemorrhagic skin lesions; hepatomegaly, anemia; thrombocytopenia; bone marrow involvement.
Segen's Medical Dictionary. © 2012 Farlex, Inc. All rights reserved.

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
McGraw-Hill Concise Dictionary of Modern Medicine. © 2002 by The McGraw-Hill Companies, Inc.
References in periodicals archive ?
Diabetes Insipidus and Sclerosing Cholangitis in a child may be a clue to the diagnosis of Langerhans' Cell Histiocytosis: A Case Report.
The DDx of pulmonary involvement in ECD includes, interstitial pneumonia and pulmonary Langerhans' cell histiocytosis.
Natural course of isolated pulmonary Langerhans' cell histiocytosis in a toddler 3-year follow-up.
Lung transplantation for pulmonary Langerhans' cell histiocytosis: a multicenter analysis.
Purely cutaneous Langerhans' cell histiocytosis in an adult woman.
Langerhans' cell histiocytosis: report of a case with temporal localization.
Labyrinthine involvement in Langerhans' cell histiocytosis. Int J Pediatr Otorhinolaryngol 1998;46(1-2):109-15.
In addition to RDD, the differential diagnosis for recurrent, sterile osteomyelitis should include other uncommon causes such as Langerhans' cell histiocytosis (LCH) and related histiocytoses [7].
Gunhan, "Oral mucosal involvement in Langerhans' cell histiocytosis: Long-term follow-up of a rare case," Australian Dental Journal, vol.
Rosso et al., "Cathepsin D and E coexpression in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and Langerhans' cell histiocytosis: further evidences of a phenotypic overlap between these histiocytic disorders," Virchows Archiv, vol.
Adult pulmonary Langerhans' cell histiocytosis. Eur Respir J 2006;27:1272-85.

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