{"Name":"Localized Castleman disease","DiseaseID__c":"GARD:0006005","id":6005,"encodedName":"localized-castleman-disease","IsDeleted":false,"Disease_Name_Full__c":"Localized Castleman disease","Xref_IDs__c":"1156784009; C115200; C3898582; MEDGEN:858176; MONDO:0019753; ORPHA:93685","USA_Estimate__c":"50,000","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":"80,000 to 800,000","No_of_HRSA_records__c":0,"Evidence_Based_Score__c":0,"No_of_Disease_Descriptions__c":4,"Disease_Characteristics_Score__c":4,"No_of_Age_at_Onset__c":1,"Description_Source__c":"MONDO:0019753","Disease_Description__c":"A rare lymphoid hemopathy characterized by involvement of a single lymph node or a group of lymph nodes in one lymph node station (most commonly in the mediastinum, neck, abdomen, or retroperitoneum). Histopathologically, it may occur as a hyaline vascular subtype with hyaline-vascular follicles and a fibrotic and hypervascular stroma with sinus compression, or a mixed/plasmacytic subtype with dense, interfollicular sheets of (usually polytypic) plasma cells extending to the cortex, and variably sized lymphoid follicles including some with regressive changes. Clinically, most patients are asymptomatic, and lesions are detected incidentally.","GARD_Name__c":"Localized Castleman disease","GARD_Synonym__c":"localised angiofollicular lymphoid hyperplasia; localized angiofollicular lymphoid hyperplasia; unicentric castleman disease","Curated_Disease_Description_Source__c":"GARD:0006005","Curated_Disease_Description__c":"Unicentric Castleman disease (UCD) is a condition that affects the lymph nodes and related tissues. It is a form of Castleman disease that is \"localized\" to a single set of lymph nodes (as opposed to multicentric Castleman disease which has more widespread effects). UCD often starts as an enlarged lymph node. Depending on the location of this enlarged node, some people will have no additional features of the condition, while others will develop symptoms when it pushes on nearby organs and/or tissues. The exact underlying cause of UCD is currently unknown.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":"50,000","Age_at_Onset_Snippet_Text__c":"at any time in life","SourceID__c":"ORPHA:93685","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Entity","MONDO_ID__c":"MONDO:0019753","ORPHANET_ID__c":"ORPHA:93685","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Enfermedad de castleman unicéntrica","Spanish_Description_Source__c":"ORPHA:93685","Spanish_Description__c":"Es una hemopatía linfoide poco frecuente caracterizada por la afectación de un solo ganglio linfático o de un grupo de ganglios linfáticos en un linfocentro (más comúnmente en el mediastino, el cuello, el abdomen o el retroperitoneo). Histopatológicamente, puede manifestarse como un subtipo hialino-vascular con folículos hialinos vasculares y un estroma fibrótico e hipervascular con compresión de los senos paranasales, o como un subtipo mixto/plasmocitoide con láminas interfoliculares densas de células plasmáticas (generalmente politípicas) que se extienden a la corteza y folículos linfoides de tamaño variable incluyendo algunos con cambios regresivos. Clínicamente, la mayoría de los pacientes permanecen asintomáticos y las lesiones se detectan de manera incidental.","Spanish_Disease_Name__c":"enfermedad de castleman unicéntrica","Spanish_GARD_Synonym__c":"enfermedad de castleman localizada","Category_Linearization__c":"ORPHA:97992","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":"Unicentric Castleman disease (UCD) is a condition that affects the lymph nodes and related tissues. It is a form of Castleman disease that is \"localized\" to a single set of lymph nodes (as opposed to multicentric Castleman disease which has more widespread effects). UCD often starts as an enlarged lymph node. Depending on the location of this enlarged node, some people will have no additional features of the condition, while others will develop symptoms when it pushes on nearby organs and/or tissues. The exact underlying cause of UCD is currently unknown.","Curated_Disease_Description_Source__c":"GARD:0006005","GARD_Synonym__c":"localised angiofollicular lymphoid hyperplasia; localized angiofollicular lymphoid hyperplasia; unicentric castleman disease","Name":"Localized Castleman disease","Curated_USA_Estimate__c":"50,000","estimateUsa":"50,000"}],"Organization_Supported_Diseases__c":[{"Account_Name__c":"Autoimmune Association","Website__c":"https://autoimmune.org/"},{"Account_Name__c":"Castleman Disease Collaborative Network","Website__c":"https://cdcn.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"},{"Tag_Name__c":"Pediatrics","Tag_Category__c":"Specialist"}],"Age_At_Onset__c":[{"Age_At_Onset__c":"All ages","Provided_By__c":"ORPHA:93685"}],"External_Identifier_Disease__c":[{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C115200","Source__c":"C3898582; MONDO:0019753","Xref__c":"C115200"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=858176","Source__c":"C3898582","Xref__c":"MEDGEN:858176"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C3898582","Source__c":"C3898582","Xref__c":"C3898582"},{"URL__c":"https://www.orpha.net/en/disease/detail/93685","Source__c":"C3898582; MONDO:0019753; ORPHA:93685","Xref__c":"ORPHA:93685"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0019753","Source__c":"GARD:0006005","Xref__c":"MONDO:0019753"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=1156784009","Source__c":"C3898582","Xref__c":"1156784009"}],"tags":{"Specialist":["Cancer - Oncologist","Hematology","Pediatrics"],"Disease Category":["Cancer","Hematology"]},"synonyms":["localised angiofollicular lymphoid hyperplasia"," localized angiofollicular lymphoid hyperplasia"," unicentric castleman disease"]}