Langerhans' cell histiocytosis

(redirected from Multiple bone involvement)

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 ?
Clinically FD is divided into three groups (3) (1) monostotic: single bone involvement, which is most common (70%) (2) polyostotic: multiple bone involvement, a less common form (30%), and (3) McCune-Albright syndrome (MAS), a rare variant of mono/polyostotic disease in which FD is associated with cafe-au-lait macules and/or endocrinopathies (4,5).
Those patients with multiple bone involvement more frequently had concurrent septic arthritis than those with a single bone lesion.
Anaplastic large-cell lymphoma of null-cell type with multiple bone involvement.

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