{"Name":"Richter syndrome","DiseaseID__c":"GARD:0007578","id":7578,"encodedName":"richter-syndrome","IsDeleted":false,"Disease_Name_Full__c":"Richter syndrome","Xref_IDs__c":"277550009; 423022887; C0349631; C35424; DOID:1703; MEDGEN:91159; MONDO:0002083","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":3,"Disease_Characteristics_Score__c":2,"No_of_Age_at_Onset__c":0,"Description_Source__c":"MONDO:0002083","Disease_Description__c":"Transformation of chronic lymphocytic leukemia into aggressive non-Hodgkin's lymphoma, usually diffuse large B-cell lymphoma (immunoblastic or centroblastic variant). Occasional cases of transformation to Hodgkin's lymphoma have also been described, particularly in patients treated with purine nucleotide analogs. Molecular genetic studies suggest that in approximately half of the cases, the lymphoma is clonally related to the underlying chronic lymphocytic leukemia, whereas in the remaining cases the lymphoma probably represents a secondary, unrelated neoplasm.","GARD_Name__c":"Richter syndrome","GARD_Synonym__c":"richter transformation; richter's syndrome; richter's transformation","Curated_Disease_Description_Source__c":"GARD:0007578","Curated_Disease_Description__c":"Richter syndrome is a rare condition in which chronic lymphocytic leukemia (CLL) changes into a fast-growing type of lymphoma. Symptoms of Richter syndrome can include fever, loss of weight and muscle mass, abdominal pain, and enlargement of the lymph nodes, liver, and spleen. Laboratory results may show anemia and low platelet counts (which can lead to easy bleeding and bruising).","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:0002083","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":"Richter syndrome is a rare condition in which chronic lymphocytic leukemia (CLL) changes into a fast-growing type of lymphoma. Symptoms of Richter syndrome can include fever, loss of weight and muscle mass, abdominal pain, and enlargement of the lymph nodes, liver, and spleen. Laboratory results may show anemia and low platelet counts (which can lead to easy bleeding and bruising).","Curated_Disease_Description_Source__c":"GARD:0007578","GARD_Synonym__c":"richter transformation; richter's syndrome; richter's transformation","Name":"Richter syndrome","estimateUsa":""}],"Organization_Supported_Diseases__c":[{"Account_Name__c":"American Cancer Society","Website__c":"https://www.cancer.org"},{"Account_Name__c":"Lymphoma Research Foundation","Website__c":"https://lymphoma.org/"}],"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://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C35424","Source__c":"C0349631; MONDO:0002083","Xref__c":"C35424"},{"URL__c":"https://www.ebi.ac.uk/ols4/ontologies/doid/classes?obo_id=DOID%3A1703","Source__c":"MONDO:0002083","Xref__c":"DOID:1703"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=277550009","Source__c":"C0349631; MONDO:0002083","Xref__c":"277550009"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=91159","Source__c":"C0349631","Xref__c":"MEDGEN:91159"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C0349631","Source__c":"C0349631","Xref__c":"C0349631"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0002083","Source__c":"GARD:0007578","Xref__c":"MONDO:0002083"},{"URL__c":"https://secure.ssa.gov/apps10/poms.nsf/lnx/0423022887","Xref__c":"423022887"}],"tags":{"Disease Category":["Cancer"]},"synonyms":["richter transformation"," richter's syndrome"," richter's transformation"]}