{"Name":"Lymphomatoid granulomatosis","DiseaseID__c":"GARD:0006943","id":6943,"encodedName":"lymphomatoid-granulomatosis","IsDeleted":false,"Disease_Name_Full__c":"Lymphomatoid granulomatosis","Xref_IDs__c":"239940004; 789177000; C0024307; C7930; D008230; DOID:0081307; MEDGEN:6161; MONDO:0019466; ORPHA:86869","USA_Estimate__c":null,"No_of_Specialist_Tagsa__c":2,"No_of_ClinGen_records__c":0,"No_of_GeneReviews__c":0,"No_of_HHS_records__c":0,"World_Estimate__c":null,"No_of_HRSA_records__c":0,"Evidence_Based_Score__c":0,"No_of_Disease_Descriptions__c":3,"Disease_Characteristics_Score__c":3,"No_of_Age_at_Onset__c":1,"Description_Source__c":"MONDO:0019466","Disease_Description__c":"Lymphomatoid granulomatosis (LYG) is a very rare Epstein-Barr virus (EBV)-driven lymphoproliferative disease most commonly occurring in adults (in the fourth to sixth decade of life) and commonly affecting the lungs (with presentations varying from small bilateral pulmonary nodules to large necrotic and sometimes cavitating lesions), skin, central nervous system, and kidneys, but only very rarely affecting the lymph nodes and spleen. The symptoms associated with LYG depend on the site of disease involvement but mainly include cough, dyspnea or chest pain (in those with pulmonary involvement) and constitutional symptoms such as weight loss and fever.","GARD_Name__c":"Lymphomatoid granulomatosis","GARD_Synonym__c":"angiocentric immunoproliferative lesion; lg - lymphomatoid granulomatosis; lyg","Curated_Disease_Description_Source__c":"GARD:0006943","Curated_Disease_Description__c":"Lymphomatoid granulomatosis is a rare disorder characterized by an overproduction of white blood cells known as B lymphocytes. These B cells can build up in the tissues of the body, causing damage to the blood vessels. In many cases of Lymphomatoid granulomatosis, the abnormal B cells contain the Epstein-Barr virus. The disease is more common in men, usually after the fifth decade of life. Lymphomatoid granulomatosis most commonly affects the lungs, though other areas of the body may also be affected. Signs and symptoms vary but can include cough, shortness of breath, tightness of the chest, fever, weight loss, and fatigue. Skin lesions and central nervous system changes such as headaches, seizures, and ataxia may also be seen. Rarely, the disorder can affect the kidneys or liver. The cause of the disorder is not well understood, though a combination of genetic and immune factors are thought to play a part.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":null,"Age_at_Onset_Snippet_Text__c":"as an Adult","SourceID__c":"ORPHA:86869","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Grouping","MONDO_ID__c":"MONDO:0019466","ORPHANET_ID__c":"ORPHA:86869","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Granulomatosis linfomatoide","Spanish_Description_Source__c":"ORPHA:86869","Spanish_Description__c":"Es una enfermedad linfoproliferativa muy poco frecuente causada por el virus de Epstein-Barr (VEB) que se presenta con mayor frecuencia en adultos (de la cuarta a la sexta década de la vida) afectando principalmente a los pulmones (con presentaciones que varían desde pequeños nódulos pulmonares bilaterales, hasta lesiones necróticas grandes y, en ocasiones, cavitadas). La enfermedad también afecta a la piel, al sistema nervioso central, a los riñones, y, de manera excepcional, a los ganglios linfáticos y al bazo. Los síntomas asociados dependen del área afectada, pero principalmente incluyen tos, disnea o dolor de pecho (en aquellos con afectación pulmonar) y síntomas constitucionales tales como pérdida de peso y fiebre.","Spanish_Disease_Name__c":"granulomatosis linfomatoide","Spanish_GARD_Synonym__c":"lyg","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":"Lymphomatoid granulomatosis is a rare disorder characterized by an overproduction of white blood cells known as B lymphocytes. These B cells can build up in the tissues of the body, causing damage to the blood vessels. In many cases of Lymphomatoid granulomatosis, the abnormal B cells contain the Epstein-Barr virus. The disease is more common in men, usually after the fifth decade of life. Lymphomatoid granulomatosis most commonly affects the lungs, though other areas of the body may also be affected. Signs and symptoms vary but can include cough, shortness of breath, tightness of the chest, fever, weight loss, and fatigue. Skin lesions and central nervous system changes such as headaches, seizures, and ataxia may also be seen. Rarely, the disorder can affect the kidneys or liver. The cause of the disorder is not well understood, though a combination of genetic and immune factors are thought to play a part.","Curated_Disease_Description_Source__c":"GARD:0006943","GARD_Synonym__c":"angiocentric immunoproliferative lesion; lg - lymphomatoid granulomatosis; lyg","Name":"Lymphomatoid granulomatosis","estimateUsa":""}],"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":"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:86869"}],"External_Identifier_Disease__c":[{"URL__c":"https://www.ncbi.nlm.nih.gov/mesh/C008230","Source__c":"C0024307; MONDO:0019466","Xref__c":"D008230"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=6161","Source__c":"C0024307","Xref__c":"MEDGEN:6161"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=239940004","Source__c":"C0024307; MONDO:0019466","Xref__c":"239940004"},{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C7930","Source__c":"C0024307; MONDO:0019466","Xref__c":"C7930"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C0024307","Source__c":"C0024307","Xref__c":"C0024307"},{"URL__c":"https://www.orpha.net/en/disease/detail/86869","Source__c":"C0024307; MONDO:0019466; ORPHA:86869","Xref__c":"ORPHA:86869"},{"URL__c":"https://www.ebi.ac.uk/ols4/ontologies/doid/classes?obo_id=DOID%3A0081307","Source__c":"MONDO:0019466","Xref__c":"DOID:0081307"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0019466","Source__c":"GARD:0006943","Xref__c":"MONDO:0019466"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=41556003","Source__c":"C0024307","Xref__c":"41556003"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=789177000","Source__c":"C0024307","Xref__c":"789177000"},{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C40970","Source__c":"C0024307","Xref__c":"C40970"}],"tags":{"Specialist":["Cancer - Oncologist","Hematology"],"Disease Category":["Cancer","Hematology"]},"synonyms":["angiocentric immunoproliferative lesion"," lg - lymphomatoid granulomatosis"," lyg"]}