{"Name":"CD4+/CD56+ hematodermic neoplasm","DiseaseID__c":"GARD:0010556","id":10556,"encodedName":"cd4cd56-hematodermic-neoplasm","IsDeleted":false,"Disease_Name_Full__c":"CD4+/CD56+ hematodermic neoplasm","Xref_IDs__c":"423022117; 445105005; C1301363; C7203; C86.4; DOID:0081076; MEDGEN:220972; MONDO:0019467; ORPHA:86870","USA_Estimate__c":"200,000","No_of_Specialist_Tagsa__c":2,"No_of_ClinGen_records__c":0,"No_of_GeneReviews__c":0,"No_of_HHS_records__c":1,"World_Estimate__c":"800,000 to 5,000,000","No_of_HRSA_records__c":0,"Evidence_Based_Score__c":1,"No_of_Disease_Descriptions__c":3,"Disease_Characteristics_Score__c":4,"No_of_Age_at_Onset__c":1,"Description_Source__c":"MONDO:0019467","Disease_Description__c":"An aggressive immature hematologic neoplasm formerly known as blastic NK cell lymphoma, composed of cells with a lymphoblast-like morphology. Recent evidence suggests derivation from a plasmacytoid monocyte. Patients present with cutaneous tumors and bone marrow involvement.","GARD_Name__c":"CD4+/CD56+ hematodermic neoplasm","GARD_Synonym__c":"agranular cd4+ cd56+ hematodermic neoplasm/tumor; agranular cd4+ natural killer cell leukaemia; agranular cd4+ natural killer cell leukemia; blastic natural killer leukemia/lymphoma; blastic nk-cell lymphoma; blastic plasmacytoid dendritic cell; blastic plasmacytoid dendritic cell neoplasm; bpdcn; early plasmacytoid dendritic cell leukemia/lymphoma; lymphoblastoid variant of nk-cell lymphoma; malignant blastic plasmacytoid dendritic cell neoplasm; monomorphic nk-cell lymphoma; primary cutaneous cd4+/cd56+ hematolymphoid neoplasm","Curated_Disease_Description_Source__c":"MONDO:0019467","Curated_Disease_Description__c":"An aggressive immature hematologic neoplasm formerly known as blastic NK cell lymphoma, composed of cells with a lymphoblast-like morphology. Recent evidence suggests derivation from a plasmacytoid monocyte. Patients present with cutaneous tumors and bone marrow involvement.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":"200,000","Age_at_Onset_Snippet_Text__c":"as an Older Adult","SourceID__c":"ORPHA:86870","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Entity","MONDO_ID__c":"MONDO:0019467","ORPHANET_ID__c":"ORPHA:86870","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Neoplasia blástica de células dendríticas plasmocitoides","Spanish_Description_Source__c":null,"Spanish_Description__c":null,"Spanish_Disease_Name__c":"neoplasia blástica de células dendríticas plasmocitoides","Spanish_GARD_Synonym__c":"bpdcn","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":"An aggressive immature hematologic neoplasm formerly known as blastic NK cell lymphoma, composed of cells with a lymphoblast-like morphology. Recent evidence suggests derivation from a plasmacytoid monocyte. Patients present with cutaneous tumors and bone marrow involvement.","Curated_Disease_Description_Source__c":"MONDO:0019467","GARD_Synonym__c":"agranular cd4+ cd56+ hematodermic neoplasm/tumor; agranular cd4+ natural killer cell leukaemia; agranular cd4+ natural killer cell leukemia; blastic natural killer leukemia/lymphoma; blastic nk-cell lymphoma; blastic plasmacytoid dendritic cell; blastic plasmacytoid dendritic cell neoplasm; bpdcn; early plasmacytoid dendritic cell leukemia/lymphoma; lymphoblastoid variant of nk-cell lymphoma; malignant blastic plasmacytoid dendritic cell neoplasm; monomorphic nk-cell lymphoma; primary cutaneous cd4+/cd56+ hematolymphoid neoplasm","Name":"CD4+/CD56+ hematodermic neoplasm","Curated_USA_Estimate__c":"200,000","estimateUsa":"200,000"}],"Organization_Supported_Diseases__c":[{"Account_Name__c":"HealthTree Foundation","Website__c":"https://healthtree.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":"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":"Elderly","Provided_By__c":"ORPHA:86870"}],"External_Identifier_Disease__c":[{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=220972","Source__c":"C1301363","Xref__c":"MEDGEN:220972"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=445105005","Source__c":"C1301363; MONDO:0019467","Xref__c":"445105005"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C1301363","Source__c":"C1301363","Xref__c":"C1301363"},{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C7203","Source__c":"C1301363; MONDO:0019467","Xref__c":"C7203"},{"URL__c":"https://www.orpha.net/en/disease/detail/86870","Source__c":"C1301363; MONDO:0019467; ORPHA:86870","Xref__c":"ORPHA:86870"},{"URL__c":"https://www.ebi.ac.uk/ols4/ontologies/doid/classes?obo_id=DOID%3A0081076","Source__c":"MONDO:0019467","Xref__c":"DOID:0081076"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0019467","Source__c":"GARD:0010556","Xref__c":"MONDO:0019467"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=445030005","Source__c":"C1301363","Xref__c":"445030005"},{"URL__c":"http://purl.bioontology.org/ontology/ICD10CM/C86.4","Source__c":"MONDO:0019467","Xref__c":"C86.4"},{"URL__c":"https://www.ncbi.nlm.nih.gov/mesh/C000099067","Source__c":"C1301363","Xref__c":"D000099067"},{"URL__c":"https://secure.ssa.gov/apps10/poms.nsf/lnx/0423022117","Xref__c":"423022117"}],"tags":{"Specialist":["Cancer - Oncologist","Hematology"],"Disease Category":["Cancer","Hematology"]},"synonyms":["agranular cd4+ cd56+ hematodermic neoplasm/tumor"," agranular cd4+ natural killer cell leukaemia"," agranular cd4+ natural killer cell leukemia"," blastic natural killer leukemia/lymphoma"," blastic nk-cell lymphoma"," blastic plasmacytoid dendritic cell"," blastic plasmacytoid dendritic cell neoplasm"," bpdcn"," early plasmacytoid dendritic cell leukemia/lymphoma"," lymphoblastoid variant of nk-cell lymphoma"," malignant blastic plasmacytoid dendritic cell neoplasm"," monomorphic nk-cell lymphoma"," primary cutaneous cd4+/cd56+ hematolymphoid neoplasm"]}