Langerhans' cell histiocytosis

(redirected from Multiple organ 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 ?
Multiple organ involvement in children at the time of diagnosis may be less common than in adults.
Since her youth,McElgunn has had Behcet's disease, a form of vasculitis, which manifests with ocular, oral, genital and skin lesions as well as arthritis and multiple organ involvement.
Finally, seeking for possible cystic lesions at other sites in the body is required since multiple organ involvement is possible (3).
In conclusion, malaria pancreatitis, though rare, is usually associated with multiple organ involvement.
These tumors usually involve single organs, but multiple organ involvement has also been reported.
Rickettsial diseases have been reported from various regions of India in the recent past (4-6); in the past few years, acute febrile illness with multiple organ involvement was diagnosed in several patients in our area.
5%), multiple organ involvement (>50%), and doxorubicin-resistance (55.

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