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