{"Name":"Grade III lymphomatoid granulomatosis","DiseaseID__c":"GARD:0026761","id":26761,"encodedName":"grade-iii-lymphomatoid-granulomatosis","IsDeleted":false,"Disease_Name_Full__c":"Grade III lymphomatoid granulomatosis","Xref_IDs__c":"423022800; C0279067; C7933; DOID:0081310; MEDGEN:78928; MONDO:0859749","USA_Estimate__c":null,"No_of_Specialist_Tagsa__c":0,"No_of_ClinGen_records__c":0,"No_of_GeneReviews__c":0,"No_of_HHS_records__c":1,"World_Estimate__c":null,"No_of_HRSA_records__c":0,"Evidence_Based_Score__c":1,"No_of_Disease_Descriptions__c":2,"Disease_Characteristics_Score__c":1,"No_of_Age_at_Onset__c":0,"Description_Source__c":"MONDO:0859749","Disease_Description__c":"A lymphomatoid granulomatosis that is characterized by the presence of aggregates of neoplastic large B-lymphocytes, usually admixed with pleomorphic and Hodgkin-like cells, in a background of chronic inflammation. Necrotic changes are present and are usually extensive.","GARD_Name__c":"Grade III lymphomatoid granulomatosis","GARD_Synonym__c":null,"Curated_Disease_Description_Source__c":"MONDO:0859749","Curated_Disease_Description__c":"Lymphomatoid granulomatosis (LG) is a rare, progressive malignant neoplastic disease in adults and children (although it is most common in individuals in the fifth and sixth decade), where nodular lesions destroy blood vessels. In addition, the lungs are usually affected. LG is composed of B-cells positive for Epstein-Barr virus (EBV) and mixed with reactive T-cells. It often occurs in association with an underlying immunodeficiency state such as rheumatoid arthritis, organ transplantation, and human immune deficiency virus (HIV) infection. A grading system from Grade I to Grade III for LG is based on the number of atypical lymphocytes, EBV-positive B-cells, and amount of tissue destruction (necrosis). The advanced form (grade III) of LG is approached clinically as a subtype of diffuse large B-cell non-Hodgkin lymphoma (NHL). The clinical features reflect systemic multi-organ disease with lung, skin, and central nervous system (CNS) involvement.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":null,"Age_at_Onset_Snippet_Text__c":null,"SourceID__c":null,"Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Entity","MONDO_ID__c":"MONDO:0859749","ORPHANET_ID__c":null,"Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":null,"Spanish_Description_Source__c":null,"Spanish_Description__c":null,"Spanish_Disease_Name__c":null,"Spanish_GARD_Synonym__c":null,"Category_Linearization__c":null,"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 (LG) is a rare, progressive malignant neoplastic disease in adults and children (although it is most common in individuals in the fifth and sixth decade), where nodular lesions destroy blood vessels. In addition, the lungs are usually affected. LG is composed of B-cells positive for Epstein-Barr virus (EBV) and mixed with reactive T-cells. It often occurs in association with an underlying immunodeficiency state such as rheumatoid arthritis, organ transplantation, and human immune deficiency virus (HIV) infection. A grading system from Grade I to Grade III for LG is based on the number of atypical lymphocytes, EBV-positive B-cells, and amount of tissue destruction (necrosis). The advanced form (grade III) of LG is approached clinically as a subtype of diffuse large B-cell non-Hodgkin lymphoma (NHL). The clinical features reflect systemic multi-organ disease with lung, skin, and central nervous system (CNS) involvement.","Curated_Disease_Description_Source__c":"MONDO:0859749","Name":"Grade III lymphomatoid granulomatosis","estimateUsa":""}],"GARD_Disease_Tag__c":[{"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"}],"External_Identifier_Disease__c":[{"URL__c":"https://www.ebi.ac.uk/ols4/ontologies/doid/classes?obo_id=DOID%3A0081310","Source__c":"MONDO:0859749","Xref__c":"DOID:0081310"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C0279067","Source__c":"C0279067","Xref__c":"C0279067"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=78928","Source__c":"C0279067","Xref__c":"MEDGEN:78928"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0859749","Source__c":"GARD:0026761","Xref__c":"MONDO:0859749"},{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C7933","Source__c":"C0279067; MONDO:0859749","Xref__c":"C7933"},{"URL__c":"https://secure.ssa.gov/apps10/poms.nsf/lnx/0423022800","Xref__c":"423022800"}],"tags":{"Disease Category":["Cancer"]},"synonyms":[""]}