loading...| Preferred Name |
Langerhans Cell Histiocytosis |
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| Synonyms |
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| Definitions |
A neoplastic proliferation of Langerhans cells which contain Birbeck granules by ultrastructural examination. Three major overlapping syndromes are recognized: eosinophilic granuloma, Letterer-Siwe disease, and Hand-Schuller-Christian disease. The clinical course is generally related to the number of organs affected at presentation. (WHO, 2001) |
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| ID |
http://purl.obolibrary.org/obo/NCIT_C3107 |
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| ALT_DEFINITION |
Tissue infiltration by Langerhans cells (histiocytes). When infiltration involves the pituitary stalk or gland, presentation includes diabetes insipidus, which is sometimes accompanied by other pituitary dysfunction. A group of rare disorders in which too many Langerhans cells (a type of white blood cell) grow in certain tissues and organs including the bones, skin, and lungs, and damage them. Langerhans cell histiocytosis may also affect the pituitary gland (which makes hormones that control other glands and many body functions, especially growth). Langerhans cell histiocytosis is most common in children and young adults. |
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| code |
C3107 |
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| Contributing_Source |
Cellosaurus CTEP CTRP GDC NICHD |
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| definition |
A neoplastic proliferation of Langerhans cells which contain Birbeck granules by ultrastructural examination. Three major overlapping syndromes are recognized: eosinophilic granuloma, Letterer-Siwe disease, and Hand-Schuller-Christian disease. The clinical course is generally related to the number of organs affected at presentation. (WHO, 2001) |
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| Display_Name |
Langerhans Cell Histiocytosis |
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| ICD-O-3_Code |
9751/1 9751/3 9752/1 9753/1 9754/3 |
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| in_subset |
http://purl.obolibrary.org/obo/NCIT_C168658 http://purl.obolibrary.org/obo/NCIT_C168656 http://purl.obolibrary.org/obo/NCIT_C168657 http://purl.obolibrary.org/obo/NCIT_C168655 http://purl.obolibrary.org/obo/NCIT_C90259 http://purl.obolibrary.org/obo/NCIT_C103078 http://purl.obolibrary.org/obo/NCIT_C102905 http://purl.obolibrary.org/obo/NCIT_C168662 http://purl.obolibrary.org/obo/NCIT_C168661 http://purl.obolibrary.org/obo/NCIT_C177537 http://purl.obolibrary.org/obo/ncit#oncotree_slim http://purl.obolibrary.org/obo/NCIT_C116977 http://purl.obolibrary.org/obo/NCIT_C118467 http://purl.obolibrary.org/obo/NCIT_C126659 http://purl.obolibrary.org/obo/NCIT_C165258 |
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| Is_Value_For_GDC_Property | ||
| label |
Langerhans Cell Histiocytosis |
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| Legacy Concept Name |
Langerhans_Cell_Histiocytosis |
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| Maps_To |
Langerhans cell granulomatosis Langerhans cell histiocytosis, NOS Histiocytosis X, NOS 9751/1 9751/3 |
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| Neoplastic_Status |
Undetermined |
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| Preferred_Name |
Langerhans Cell Histiocytosis |
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| prefixIRI |
NCIT:C3107 |
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| prefLabel |
Langerhans Cell Histiocytosis |
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| Semantic_Type |
Neoplastic Process |
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| UMLS_CUI |
C0019621 |
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| subClassOf |