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
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.
Temporal Bone Histopathology Case of the Month: Langerhans' Cell Histiocytosis of the Temporal Bone.

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