{"Name":"Primary central nervous system lymphoma","DiseaseID__c":"GARD:0009318","id":9318,"encodedName":"primary-central-nervous-system-lymphoma","IsDeleted":false,"Disease_Name_Full__c":"Primary central nervous system lymphoma","Xref_IDs__c":"307649006; 423022640; C0280803; C9301; DOID:3234; HP:0030069; MEDGEN:79187; MONDO:0002571; ORPHA:46135","USA_Estimate__c":"5,000","No_of_Specialist_Tagsa__c":3,"No_of_ClinGen_records__c":0,"No_of_GeneReviews__c":0,"No_of_HHS_records__c":3,"World_Estimate__c":"8,000 to 80,000","No_of_HRSA_records__c":0,"Evidence_Based_Score__c":3,"No_of_Disease_Descriptions__c":4,"Disease_Characteristics_Score__c":4,"No_of_Age_at_Onset__c":2,"Description_Source__c":"MONDO:0002571","Disease_Description__c":"A non-Hodgkin or Hodgkin lymphoma that arises in the brain or spinal cord as a primary lesion. There is no evidence of lymphoma outside the central nervous system at the time of diagnosis.","GARD_Name__c":"Primary central nervous system lymphoma","GARD_Synonym__c":"central nervous system lymphoma; cns lymphoma; lymphoma of central nervous system; malignant lymphomas of cns; malignant lymphomas of the cns; microglioma; pcnsl; pcnsl - primary cns lymphoma; primary brain lymphoma; primary cns lymphoma","Curated_Disease_Description_Source__c":"GARD:0009318","Curated_Disease_Description__c":"Primary central nervous system lymphoma (primary CNS lymphoma) is a rare form of non-Hodgkin lymphoma in which cancerous cells develop in the lymph tissue of the brain and/or spinal cord. Because the eye is so close to the brain, primary CNS lymphoma can also start in the eye (called ocular lymphoma). The symptoms vary based on which parts of the central nervous system are affected, but may include nausea and vomiting; seizures; headaches; arm or leg weakness; confusion; double vision and/or hearing loss. The exact underlying cause of primary CNS lymphoma is poorly understood. However, people with a weakened immune system (such as those with acquired immunodeficiency syndrome) or who have had an organ transplant appear to have an increased risk of developing the condition.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":"5,000","Age_at_Onset_Snippet_Text__c":"as an Adult and as an Older Adult","SourceID__c":"ORPHA:46135","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Grouping","MONDO_ID__c":"MONDO:0002571","ORPHANET_ID__c":"ORPHA:46135","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Linfoma primario del sistema nervioso central","Spanish_Description_Source__c":null,"Spanish_Description__c":null,"Spanish_Disease_Name__c":"linfoma primario del sistema nervioso central","Spanish_GARD_Synonym__c":"linfoma cerebral primario; linfoma del snc primario; pcnsl","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":"Primary central nervous system lymphoma (primary CNS lymphoma) is a rare form of non-Hodgkin lymphoma in which cancerous cells develop in the lymph tissue of the brain and/or spinal cord. Because the eye is so close to the brain, primary CNS lymphoma can also start in the eye (called ocular lymphoma). The symptoms vary based on which parts of the central nervous system are affected, but may include nausea and vomiting; seizures; headaches; arm or leg weakness; confusion; double vision and/or hearing loss. The exact underlying cause of primary CNS lymphoma is poorly understood. However, people with a weakened immune system (such as those with acquired immunodeficiency syndrome) or who have had an organ transplant appear to have an increased risk of developing the condition.","Curated_Disease_Description_Source__c":"GARD:0009318","GARD_Synonym__c":"central nervous system lymphoma; cns lymphoma; lymphoma of central nervous system; malignant lymphomas of cns; malignant lymphomas of the cns; microglioma; pcnsl; pcnsl - primary cns lymphoma; primary brain lymphoma; primary cns lymphoma","Name":"Primary central nervous system lymphoma","Curated_USA_Estimate__c":"5,000","estimateUsa":"5,000"}],"Organization_Supported_Diseases__c":[{"Account_Name__c":"Lymphoma Research Foundation","Website__c":"https://lymphoma.org/"},{"Account_Name__c":"Blood Cancer United","Website__c":"https://bloodcancerunited.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":"Neurology","Tag_Category__c":"Disease Category;Specialist","category_description":"Neurological diseases affect the brain, spinal cord, cranial nerves, autonomic nerves, or other peripheral nerves.","curated_tag_name":"Neurological diseases"},{"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":"Adult","Provided_By__c":"ORPHA:46135"},{"Age_At_Onset__c":"Elderly","Provided_By__c":"ORPHA:46135"}],"External_Identifier_Disease__c":[{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C9301","Source__c":"C0280803; MONDO:0002571","Xref__c":"C9301"},{"URL__c":"https://www.orpha.net/en/disease/detail/46135","Source__c":"C0280803; MONDO:0002571; ORPHA:46135","Xref__c":"ORPHA:46135"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C0280803","Source__c":"C0280803","Xref__c":"C0280803"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=307649006","Source__c":"C0280803; MONDO:0002571","Xref__c":"307649006"},{"URL__c":"https://www.ebi.ac.uk/ols4/ontologies/doid/classes?obo_id=DOID%3A3234","Source__c":"MONDO:0002571","Xref__c":"DOID:3234"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=79187","Source__c":"C0280803","Xref__c":"MEDGEN:79187"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0002571","Source__c":"GARD:0009318","Xref__c":"MONDO:0002571"},{"URL__c":"https://hpo.jax.org/browse/term/HP:0030069","Source__c":"C0280803","Xref__c":"HP:0030069"},{"URL__c":"https://secure.ssa.gov/apps10/poms.nsf/lnx/0423022640","Xref__c":"423022640"},{"URL__c":"https://www.cancer.gov/types/lymphoma"},{"URL__c":"https://www.cancer.gov/types/lymphoma/patient/primary-cns-lymphoma-treatment-pdq"}],"tags":{"Specialist":["Cancer - Oncologist","Neurology","Hematology"],"Disease Category":["Cancer","Neurology","Hematology"]},"synonyms":["central nervous system lymphoma"," cns lymphoma"," lymphoma of central nervous system"," malignant lymphomas of cns"," malignant lymphomas of the cns"," microglioma"," pcnsl"," pcnsl - primary cns lymphoma"," primary brain lymphoma"," primary cns lymphoma"]}