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 ?
Interpretation of multiple organ involvement by CT and/or MRI and getting acquainted with these imaging findings is essential for early diagnosis and prompt management.
She was diagnosed as DRESS syndrome caused by the CM and etodolac due to (1) the close relationship between the initiation of CM and etodolac and the onset of symptoms, (2) the elimination rate of CM and etodolac in elderly patients are reduced related to the reduction in renal function and aging,[sup][6] (3) the risk of acute nephrotoxicity of NSAIDs increases with age,[sup][7] (4) fever, (5) skin rash with facial edema, (6) typical laboratory tests included the presence of eosinophilia and atypical lymphocytes, (7) a positive finding of CMV infection which is associated with the development of DRESS, (8) multiple organ involvement including the liver, kidney, lung, and pancreas, and (9) persistent and worsening symptoms after withdrawal from the causative drug.
Conclusion: TS is a rare disease in newborns and occurs with multiple organ involvement.
The data showed that patients with NTM BSI were mostly immunocompromised and usually had multiple organ involvement.
The clinical presentation of cSLE is frequently more severe than adult onset SLE with multiple organ involvement, particularly the kidney and central nervous system.
Multiple organ involvement in Perinatal asphyxia, J Pediatr 1995, 127; 786-793.
Multiple organ involvement in children at the time of diagnosis may be less common than in adults.
6] Multi-system disease consists of various combinations of multiple organ involvement, and there may also be organ dysfunction and pronounced generalised symptoms.
Scrofuloderma with multiple organ involvement in a 5-year-old child.
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.

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