{"Name":"T-cell/histiocyte rich large B cell lymphoma","DiseaseID__c":"GARD:0012257","id":12257,"encodedName":"t-cellhistiocyte-rich-large-b-cell-lymphoma","IsDeleted":false,"Disease_Name_Full__c":"T-cell/histiocyte rich large B cell lymphoma","Xref_IDs__c":"C1321547; C9496; MEDGEN:232943; MONDO:0017597; ORPHA:300857","USA_Estimate__c":"1,000","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":"1 to 8,000","No_of_HRSA_records__c":0,"Evidence_Based_Score__c":0,"No_of_Disease_Descriptions__c":2,"Disease_Characteristics_Score__c":3,"No_of_Age_at_Onset__c":4,"Description_Source__c":"MONDO:0017597","Disease_Description__c":"T-cell/histiocyte rich large B cell lymphoma (THRLBCL) is a rare variant of diffuse large B-cell lymphoma (DLBCL; see this term), mainly affecting middle-aged men and often not being discovered until an advanced disease stage, with involvement of the spleen, liver and bone marrow occurring at a greater frequency than in DLBCL. It is often difficult to diagnose due to its similarity with other lymphoid diseases such as classic Hodgkin lymphoma and nodular lymphocyte-predominant Hodgkin lymphoma (see these terms) and has an aggressive clinical course.","GARD_Name__c":"T-cell/histiocyte rich large B cell lymphoma","GARD_Synonym__c":"histiocyte-rich large b-cell lymphoma; t-cell histiocyte rich large b-cell lymphoma; t-cell hystiocyte rich large b-cell lymphoma; t-cell rich large b-cell lymphoma; t-cell rich/histiocyte-rich large b-cell lymphoma; t-cell/histiocyte rich large b-cell lymphoma; t-cell/histiocyte rich lymphoma; t-cell/histiocyte-rich large b-cell lymphoma; thrlbcl","Curated_Disease_Description_Source__c":"MONDO:0017597","Curated_Disease_Description__c":"T-cell/histiocyte rich large B cell lymphoma (THRLBCL) is a rare variant of diffuse large B-cell lymphoma (DLBCL; see this term), mainly affecting middle-aged men and often not being discovered until an advanced disease stage, with involvement of the spleen, liver and bone marrow occurring at a greater frequency than in DLBCL. It is often difficult to diagnose due to its similarity with other lymphoid diseases such as classic Hodgkin lymphoma and nodular lymphocyte-predominant Hodgkin lymphoma (see these terms) and has an aggressive clinical course.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":"1,000","Age_at_Onset_Snippet_Text__c":"at a variety of ages","SourceID__c":"ORPHA:300857","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Entity","MONDO_ID__c":"MONDO:0017597","ORPHANET_ID__c":"ORPHA:300857","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Linfoma b de células grandes rico en células -t/histiocitos","Spanish_Description_Source__c":"ORPHA:300857","Spanish_Description__c":"Es una variante poco frecuente del linfoma B difuso de células grandes (DLBCL) que afecta fundamentalmente a varones de mediana edad. Por lo general, no se diagnostica hasta una etapa avanzada de la enfermedad, con afectación del bazo, hígado y médula ósea con mayor frecuencia que en el DLBCL. A menudo, el diagnóstico resulta difícil debido a su similitud con otras enfermedades linfoides, tales como el linfoma de Hodgkin clásico y el linfoma de Hodgkin con predominio de linfocitos nodulares. Tiene un curso clínico agresivo.","Spanish_Disease_Name__c":"linfoma b de células grandes rico en células -t/histiocitos","Spanish_GARD_Synonym__c":"thrlbcl","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":"T-cell/histiocyte rich large B cell lymphoma (THRLBCL) is a rare variant of diffuse large B-cell lymphoma (DLBCL; see this term), mainly affecting middle-aged men and often not being discovered until an advanced disease stage, with involvement of the spleen, liver and bone marrow occurring at a greater frequency than in DLBCL. It is often difficult to diagnose due to its similarity with other lymphoid diseases such as classic Hodgkin lymphoma and nodular lymphocyte-predominant Hodgkin lymphoma (see these terms) and has an aggressive clinical course.","Curated_Disease_Description_Source__c":"MONDO:0017597","GARD_Synonym__c":"histiocyte-rich large b-cell lymphoma; t-cell histiocyte rich large b-cell lymphoma; t-cell hystiocyte rich large b-cell lymphoma; t-cell rich large b-cell lymphoma; t-cell rich/histiocyte-rich large b-cell lymphoma; t-cell/histiocyte rich large b-cell lymphoma; t-cell/histiocyte rich lymphoma; t-cell/histiocyte-rich large b-cell lymphoma; thrlbcl","Name":"T-cell/histiocyte rich large B cell lymphoma","Curated_USA_Estimate__c":"1,000","estimateUsa":"1,000"}],"Organization_Supported_Diseases__c":[{"Account_Name__c":"Lymphoma Action","Website__c":"https://lymphoma-action.org.uk"}],"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"},{"Tag_Name__c":"Pediatrics","Tag_Category__c":"Specialist"}],"Age_At_Onset__c":[{"Age_At_Onset__c":"Adult","Provided_By__c":"ORPHA:300857"},{"Age_At_Onset__c":"Adolescent","Provided_By__c":"ORPHA:300857"},{"Age_At_Onset__c":"Childhood","Provided_By__c":"ORPHA:300857"},{"Age_At_Onset__c":"Elderly","Provided_By__c":"ORPHA:300857"}],"External_Identifier_Disease__c":[{"URL__c":"https://www.orpha.net/en/disease/detail/300857","Source__c":"C1321547; MONDO:0017597; ORPHA:300857","Xref__c":"ORPHA:300857"},{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C9496","Source__c":"C1321547; MONDO:0017597","Xref__c":"C9496"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=232943","Source__c":"C1321547","Xref__c":"MEDGEN:232943"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C1321547","Source__c":"C1321547","Xref__c":"C1321547"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=450959001","Source__c":"C1321547","Xref__c":"450959001"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=724645006","Source__c":"C1321547","Xref__c":"724645006"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0017597","Source__c":"GARD:0012257","Xref__c":"MONDO:0017597"}],"Inheritance__c":["Non-Mendelian inheritance"],"tags":{"Specialist":["Cancer - Oncologist","Hematology","Pediatrics"],"Disease Category":["Cancer","Hematology"]},"synonyms":["histiocyte-rich large b-cell lymphoma"," t-cell histiocyte rich large b-cell lymphoma"," t-cell hystiocyte rich large b-cell lymphoma"," t-cell rich large b-cell lymphoma"," t-cell rich/histiocyte-rich large b-cell lymphoma"," t-cell/histiocyte rich large b-cell lymphoma"," t-cell/histiocyte rich lymphoma"," t-cell/histiocyte-rich large b-cell lymphoma"," thrlbcl"]}