Biomaterial Database

Langerhans cell histiocytosis with orbital involvement. 1997

T R Kramer, and R J Noecker, and J M Miller, and L C Clark

Department of Ophthalmology, University of Arizona Health Sciences Center, Tucson 85719, USA. TKramer@eyes.Arizona.edu

OBJECTIVE To review three cases of Langerhans cell histiocytosis with orbital involvement that represent a significantly excessive incidence of this rare disease in one community. Current diagnostic criteria and therapeutic modalities related to Langerhans cell histiocytosis are reviewed. METHODS Case reports. We present clinical, radiologic, histopathologic, and epidemiologic information on three patients with Langerhans cell histiocytosis. RESULTS All three children, born within 18 months of one another, manifested rapidly progressive unilateral proptosis at age 2 years. By computed tomography, all had moderately enhancing lesions with involvement of the sphenoid bone and lateral orbit as well as the temporal lobe of the brain. All patients were treated with a combination of vincristine and prednisone, with variable resolution of their lesions. The occurrence of three cases in children born in Nogales, Arizona/ Mexico, suggests an incidence rate of 40 per million, which is approximately 26 times the expected rate (P = .0001). CONCLUSIONS The extraordinarily high incidence and the concentration of cases in both time and space of this cluster implies that Langerhans cell histiocytosis may be a sentinel disease for unusual environmental exposures.

UI	MeSH Term	Description	Entries
D007150	Immunohistochemistry	Histochemical localization of immunoreactive substances using labeled antibodies as reagents.	Immunocytochemistry,Immunogold Techniques,Immunogold-Silver Techniques,Immunohistocytochemistry,Immunolabeling Techniques,Immunogold Technics,Immunogold-Silver Technics,Immunolabeling Technics,Immunogold Silver Technics,Immunogold Silver Techniques,Immunogold Technic,Immunogold Technique,Immunogold-Silver Technic,Immunogold-Silver Technique,Immunolabeling Technic,Immunolabeling Technique,Technic, Immunogold,Technic, Immunogold-Silver,Technic, Immunolabeling,Technics, Immunogold,Technics, Immunogold-Silver,Technics, Immunolabeling,Technique, Immunogold,Technique, Immunogold-Silver,Technique, Immunolabeling,Techniques, Immunogold,Techniques, Immunogold-Silver,Techniques, Immunolabeling
D007223	Infant	A child between 1 and 23 months of age.	Infants
D008800	Mexico	A country in NORTH AMERICA, bordering the Caribbean Sea and the Gulf of Mexico, between BELIZE and the UNITED STATES, and bordering the North Pacific Ocean, between Guatemala and the UNITED STATES.
D009915	Orbit	Bony cavity that holds the eyeball and its associated tissues and appendages.	Eye Socket,Eye Sockets,Orbits,Socket, Eye,Sockets, Eye
D009916	Orbital Diseases	Diseases of the bony orbit and contents except the eyeball.	Disease, Orbital,Diseases, Orbital,Orbital Disease
D011241	Prednisone	A synthetic anti-inflammatory glucocorticoid derived from CORTISONE. It is biologically inert and converted to PREDNISOLONE in the liver.	Dehydrocortisone,delta-Cortisone,Apo-Prednisone,Cortan,Cortancyl,Cutason,Dacortin,Decortin,Decortisyl,Deltasone,Encorton,Encortone,Enkortolon,Kortancyl,Liquid Pred,Meticorten,Orasone,Panafcort,Panasol,Predni Tablinen,Prednidib,Predniment,Prednison Acsis,Prednison Galen,Prednison Hexal,Pronisone,Rectodelt,Sone,Sterapred,Ultracorten,Winpred,Acsis, Prednison
D002675	Child, Preschool	A child between the ages of 2 and 5.	Children, Preschool,Preschool Child,Preschool Children
D005260	Female		Females
D006646	Histiocytosis, Langerhans-Cell	A group of disorders resulting from the abnormal proliferation of and tissue infiltration by LANGERHANS CELLS which can be detected by their characteristic Birbeck granules (X bodies), or by monoclonal antibody staining for their surface CD1 ANTIGENS. Langerhans-cell granulomatosis can involve a single organ, or can be a systemic disorder.	Histiocytosis X,Langerhans-Cell Granulomatosis,Langerhans-Cell Histiocytosis,Hand-Schueller-Christian Disease,Hand-Schueller-Christian Syndrome,Hand-Schüller-Christian Disease,Hand-Schüller-Christian Syndrome,Hashimoto-Pritzger Disease,Histiocytosis, Generalized,Histiocytosis-X,Langerhans Cell Granulomatosis,Langerhans Cell Granulomatosis, Pulmonary,Langerhans Cell Histiocytosis,Letterer-Siwe Disease,Non-Lipid Reticuloendotheliosis,Pulmonary Histiocytosis X,Pulmonary Langerhans Cell Granulomatosis,Schueller-Christian Disease,Systemic Aleukemic Reticuloendotheliosis,Type 2 Histiocytosis,Aleukemic Reticuloendothelioses, Systemic,Aleukemic Reticuloendotheliosis, Systemic,Cell Granulomatoses, Langerhans,Cell Granulomatosis, Langerhans,Cell Histiocytoses, Langerhans,Cell Histiocytosis, Langerhans,Disease, Hand-Schueller-Christian,Disease, Hand-Schüller-Christian,Disease, Hashimoto-Pritzger,Disease, Letterer-Siwe,Disease, Schueller-Christian,Diseases, Hashimoto-Pritzger,Generalized Histiocytoses,Generalized Histiocytosis,Granulomatoses, Langerhans Cell,Granulomatosis, Langerhans Cell,Granulomatosis, Langerhans-Cell,Hand Schueller Christian Disease,Hand Schueller Christian Syndrome,Hand Schüller Christian Disease,Hand Schüller Christian Syndrome,Hashimoto Pritzger Disease,Hashimoto-Pritzger Diseases,Histiocytoses, Generalized,Histiocytoses, Langerhans Cell,Histiocytoses, Type 2,Histiocytosis X, Pulmonary,Histiocytosis, Langerhans Cell,Histiocytosis, Type 2,Langerhans Cell Granulomatoses,Langerhans Cell Histiocytoses,Letterer Siwe Disease,Non Lipid Reticuloendotheliosis,Non-Lipid Reticuloendothelioses,Reticuloendothelioses, Non-Lipid,Reticuloendothelioses, Systemic Aleukemic,Reticuloendotheliosis, Non-Lipid,Reticuloendotheliosis, Systemic Aleukemic,Schueller Christian Disease,Syndrome, Hand-Schueller-Christian,Syndrome, Hand-Schüller-Christian,Systemic Aleukemic Reticuloendothelioses,Type 2 Histiocytoses
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man