{"Name":"Extranodal nasal NK/T cell lymphoma","DiseaseID__c":"GARD:0007041","id":7041,"encodedName":"extranodal-nasal-nkt-cell-lymphoma","IsDeleted":false,"Disease_Name_Full__c":"Extranodal nasal NK/T cell lymphoma","Xref_IDs__c":"C0392788; C4684; C86.0; DOID:0080797; MEDGEN:140278; MONDO:0019472; ORPHA:86879","USA_Estimate__c":"50,000","No_of_Specialist_Tagsa__c":3,"No_of_ClinGen_records__c":0,"No_of_GeneReviews__c":0,"No_of_HHS_records__c":0,"World_Estimate__c":"80,000 to 800,000","No_of_HRSA_records__c":0,"Evidence_Based_Score__c":0,"No_of_Disease_Descriptions__c":3,"Disease_Characteristics_Score__c":5,"No_of_Age_at_Onset__c":1,"Description_Source__c":"MONDO:0019472","Disease_Description__c":"Extranodal nasal NK/T cell lymphoma (NKTCL) is a rare, malignant neoplasm mainly affecting men in the fifth decade of life, that usually arises in the nose, paranasal sinuses, orbits or upper airway, and that can present with a nasal mass, nasal bleeding, nasal obstruction, palate perforation (i.e. midline perforation of the hard palate), and mid-facial and/or upper airway destructive lesions. In advanced disease stages, which are associated with a poor prognosis, NKTCL may disseminate to other organs. A few cases of NKTCL presenting primarily in the lymph nodes have also been described.","GARD_Name__c":"Extranodal nasal NK/T cell lymphoma","GARD_Synonym__c":"angiocentric t-cell lymphoma; extranodal natural killer/t-cell lymphoma, nasal type; extranodal nk/t lymphoma-nasal; extranodal nk/t-cell lymphoma, nasal type; lethal midline granuloma; nasal t/natural killer-cell lymphoma; nasal type extranodal nk/t-cell lymphoma; natural killer cell and t-cell malignant lymphoma nasal type; nk/t-cell lymphoma; nktcl; reticulosis, malignant; t/nk-cell lymphoma","Curated_Disease_Description_Source__c":"PlainLanguagePilotV1-Sep23","Curated_Disease_Description__c":"Extranodal nasal NK/T cell lymphoma (NKTCL) is a rare type of cancer that mostly affects men in their 50s. It usually starts in the nose, sinuses, eyes, or upper airway. The symptoms of NKTCL include:a mass in the nose, nasal bleeding, and difficulty breathing through the nose. It can also cause a hole in the roof of the mouth and lesions that damage the face or upper airway. In advanced stages of the disease, NKTCL can spread to other parts of the body, which makes it harder to treat. Some cases of NKTCL start in the lymph nodes.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":"50,000","Age_at_Onset_Snippet_Text__c":"as an Adult","SourceID__c":"ORPHA:86879","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Entity","MONDO_ID__c":"MONDO:0019472","ORPHANET_ID__c":"ORPHA:86879","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Linfoma extranodal nasal de células nk/t","Spanish_Description_Source__c":"ORPHA:86879","Spanish_Description__c":"Es una neoplasia maligna poco frecuente que afecta principalmente a los varones en la quinta década de vida. Por lo general, aparece en la nariz, los senos paranasales, las órbitas o vías respiratorias superiores, y puede presentarse con una masa / sangrado / obstrucción nasal, perforación del paladar (por ej., perforación de la línea media del paladar duro) y lesiones destructivas a nivel mediofacial y/o de las vías aéreas superiores. En estadios avanzados de la enfermedad, asociados a un mal pronóstico, esta neoplasia puede diseminarse a otros órganos. También se han descrito algunos casos de este tipo de linfomas que se presentan en los ganglios linfáticos de forma primaria.","Spanish_Disease_Name__c":"linfoma extranodal nasal de células nk/t","Spanish_GARD_Synonym__c":"granuloma letal de la línea media; linfoma angiocéntrico de células t; linfoma de células nk/t; linfoma nasal de células t/natural killer; nktcl","Category_Linearization__c":"ORPHA:250908","icd10_id__c":null,"mesh_id__c":null,"omim_id__c":null,"snomed_id__c":null,"umls_id__c":null,"GARD_Disease__c":[{"Curated_Disease_Description__c":"Extranodal nasal NK/T cell lymphoma (NKTCL) is a rare type of cancer that mostly affects men in their 50s. It usually starts in the nose, sinuses, eyes, or upper airway. The symptoms of NKTCL include:a mass in the nose, nasal bleeding, and difficulty breathing through the nose. It can also cause a hole in the roof of the mouth and lesions that damage the face or upper airway. In advanced stages of the disease, NKTCL can spread to other parts of the body, which makes it harder to treat. Some cases of NKTCL start in the lymph nodes.","Curated_Disease_Description_Source__c":"PlainLanguagePilotV1-Sep23","GARD_Synonym__c":"angiocentric t-cell lymphoma; extranodal natural killer/t-cell lymphoma, nasal type; extranodal nk/t lymphoma-nasal; extranodal nk/t-cell lymphoma, nasal type; lethal midline granuloma; nasal t/natural killer-cell lymphoma; nasal type extranodal nk/t-cell lymphoma; natural killer cell and t-cell malignant lymphoma nasal type; nk/t-cell lymphoma; nktcl; reticulosis, malignant; t/nk-cell lymphoma","Name":"Extranodal nasal NK/T cell lymphoma","Curated_USA_Estimate__c":"50,000","estimateUsa":"50,000"}],"Organization_Supported_Diseases__c":[{"Account_Name__c":"HealthTree Foundation","Website__c":"https://healthtree.org/"},{"Account_Name__c":"Lymphoma Research Foundation","Website__c":"https://lymphoma.org/"},{"Account_Name__c":"American Cancer Society","Website__c":"https://www.cancer.org"}],"GARD_Disease_Tag__c":[{"Tag_Name__c":"Cancer - Oncologist","Tag_Category__c":"Specialist"},{"Tag_Name__c":"Cancer","Tag_Category__c":"Disease Category","category_description":"Cancer is a disease in which some of the body's cells grow uncontrollably and can spread to other parts of the body.","curated_tag_name":"Cancer"},{"Tag_Name__c":"Dermatology","Tag_Category__c":"Account;Disease Category;Specialist","category_description":"Skin diseases, or integumentary system diseases, affect the skin, hair, nails, sweat glands, or oil glands.","curated_tag_name":"Skin diseases"},{"Tag_Name__c":"Hematology","Tag_Category__c":"Disease Category;Specialist","category_description":"Blood diseases affect the blood or blood-forming organs, including red blood cells, white blood cells, platelets, plasma, and bone marrow.","curated_tag_name":"Blood diseases"}],"Age_At_Onset__c":[{"Age_At_Onset__c":"Adult","Provided_By__c":"ORPHA:86879"}],"External_Identifier_Disease__c":[{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=140278","Source__c":"C0392788","Xref__c":"MEDGEN:140278"},{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C4684","Source__c":"C0392788; MONDO:0019472","Xref__c":"C4684"},{"URL__c":"https://www.ebi.ac.uk/ols4/ontologies/doid/classes?obo_id=DOID%3A0080797","Source__c":"MONDO:0019472","Xref__c":"DOID:0080797"},{"URL__c":"https://www.orpha.net/en/disease/detail/86879","Source__c":"C0392788; MONDO:0019472; ORPHA:86879","Xref__c":"ORPHA:86879"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C0392788","Source__c":"C0392788","Xref__c":"C0392788"},{"URL__c":"http://purl.bioontology.org/ontology/ICD10CM/C86.0","Source__c":"MONDO:0019472","Xref__c":"C86.0"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0019472","Source__c":"GARD:0007041","Xref__c":"MONDO:0019472"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=414166008","Source__c":"C0392788","Xref__c":"414166008"}],"Inheritance__c":["Non-Mendelian inheritance"],"tags":{"Specialist":["Cancer - Oncologist","Dermatology","Hematology"],"Disease Category":["Cancer","Dermatology","Hematology"],"Account":["Dermatology"]},"synonyms":["angiocentric t-cell lymphoma"," extranodal natural killer/t-cell lymphoma, nasal type"," extranodal nk/t lymphoma-nasal"," extranodal nk/t-cell lymphoma, nasal type"," lethal midline granuloma"," nasal t/natural killer-cell lymphoma"," nasal type extranodal nk/t-cell lymphoma"," natural killer cell and t-cell malignant lymphoma nasal type"," nk/t-cell lymphoma"," nktcl"," reticulosis, malignant"," t/nk-cell lymphoma"]}