{"Name":"Mantle cell lymphoma","DiseaseID__c":"GARD:0006969","id":6969,"encodedName":"mantle-cell-lymphoma","IsDeleted":false,"Disease_Name_Full__c":"Mantle cell lymphoma","Xref_IDs__c":"423022230; 443487006; 74654000; C4337; C4721414; C83.1; D020522; DOID:0050746; MEDGEN:1668377; MONDO:0018876; ORPHA:52416","USA_Estimate__c":"50,000","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":"80,000 to 800,000","No_of_HRSA_records__c":0,"Evidence_Based_Score__c":1,"No_of_Disease_Descriptions__c":3,"Disease_Characteristics_Score__c":5,"No_of_Age_at_Onset__c":1,"Description_Source__c":"MONDO:0018876","Disease_Description__c":"Mantle cell lymphoma is a rare form of malignant non-Hodgkin lymphoma (see this term) affecting B lymphocytes in the lymph nodes in a region called the ``mantle zone''.","GARD_Name__c":"Mantle cell lymphoma","GARD_Synonym__c":"classical mantle cell lymphoma; lcm; mantle zone lymphoma; mcl","Curated_Disease_Description_Source__c":"GARD:0006969","Curated_Disease_Description__c":"Mantle cell lymphoma (MCL) belongs to a group of diseases known as non-Hodgkin's lymphomas (NHL). NHL's are cancers that affect the the lymphatic system (part of the immune system). In MCL, there are cancerous B-cells (a type of immune system cell). The cancerous B-cells are within a region of the lymph node known as the mantle zone. Although MCLs are slow-growing cancers, the cancer is usually widespread by the time it is diagnosed.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":"50,000","Age_at_Onset_Snippet_Text__c":"as an Adult","SourceID__c":"ORPHA:52416","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Grouping","MONDO_ID__c":"MONDO:0018876","ORPHANET_ID__c":"ORPHA:52416","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Linfoma de células del manto","Spanish_Description_Source__c":"ORPHA:52416","Spanish_Description__c":"El linfoma de células del manto es una forma rara del linfoma maligno no Hodgkin (ver este término), que afecta a los linfocitos B en una región de los ganglios linfáticos llamada ``zona del manto''.","Spanish_Disease_Name__c":"linfoma de células del manto","Spanish_GARD_Synonym__c":"lcm; linfoma de la zona del manto; mcl","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":"Mantle cell lymphoma (MCL) belongs to a group of diseases known as non-Hodgkin's lymphomas (NHL). NHL's are cancers that affect the the lymphatic system (part of the immune system). In MCL, there are cancerous B-cells (a type of immune system cell). The cancerous B-cells are within a region of the lymph node known as the mantle zone. Although MCLs are slow-growing cancers, the cancer is usually widespread by the time it is diagnosed.","Curated_Disease_Description_Source__c":"GARD:0006969","GARD_Synonym__c":"classical mantle cell lymphoma; lcm; mantle zone lymphoma; mcl","Name":"Mantle cell lymphoma","Curated_USA_Estimate__c":"50,000","estimateUsa":"50,000"}],"Organization_Supported_Diseases__c":[{"Account_Name__c":"HealthTree Foundation","Website__c":"https://healthtree.org/"},{"Account_Name__c":"Smart Patients","Website__c":"https://www.smartpatients.com/"},{"Account_Name__c":"American Cancer Society","Website__c":"https://www.cancer.org"},{"Account_Name__c":"Lymphoma Research Foundation","Website__c":"https://lymphoma.org/"},{"Account_Name__c":"Blood Cancer United","Website__c":"https://bloodcancerunited.org/"},{"Account_Name__c":"Lymphoma Coalition","Website__c":"https://lymphomacoalition.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":"Adult","Provided_By__c":"ORPHA:52416"}],"External_Identifier_Disease__c":[{"URL__c":"https://raresource.nih.gov/diseases/filter/0006969","Source__c":"RareSource"},{"URL__c":"https://www.ncbi.nlm.nih.gov/mesh/C020522","Source__c":"C4721414; MONDO:0018876","Xref__c":"D020522"},{"URL__c":"https://www.ebi.ac.uk/ols4/ontologies/doid/classes?obo_id=DOID%3A0050746","Source__c":"MONDO:0018876","Xref__c":"DOID:0050746"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=1668377","Source__c":"C4721414","Xref__c":"MEDGEN:1668377"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C4721414","Source__c":"C4721414","Xref__c":"C4721414"},{"URL__c":"https://www.orpha.net/en/disease/detail/52416","Source__c":"C4721414; MONDO:0018876; ORPHA:52416","Xref__c":"ORPHA:52416"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=443487006","Source__c":"C4721414; MONDO:0018876","Xref__c":"443487006"},{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C4337","Source__c":"C4721414; MONDO:0018876","Xref__c":"C4337"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0018876","Source__c":"GARD:0006969","Xref__c":"MONDO:0018876"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=74654000","Source__c":"C4721414","Xref__c":"74654000"},{"URL__c":"http://purl.bioontology.org/ontology/ICD10CM/C83.1","Source__c":"MONDO:0018876","Xref__c":"C83.1"},{"URL__c":"https://secure.ssa.gov/apps10/poms.nsf/lnx/0423022230","Xref__c":"423022230"}],"GARD_Disease_Gene__c":[{"GeneSymbol__c":"ATM","GHR_URL__c":"https://medlineplus.gov/genetics/gene/atm","Gene_Type__c":"protein-coding gene","Causal_Gene__c":true},{"GeneSymbol__c":"CCND1","Gene_Type__c":"protein-coding gene","Causal_Gene__c":true},{"GeneSymbol__c":"IGH","Gene_Type__c":"protein-coding gene","Causal_Gene__c":true}],"Inheritance__c":["Non-Mendelian inheritance"],"GARD_Disease_Feature__c":[{"Provided_By__c":"ORPHA:52416","HPO_Frequency__c":"Frequent (30-79%)","Feature__r":{"HPO_Description__c":"Reduction of total body weight.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0001824","HPO_Synonym__c":"Loss of weight","HPO_Name__c":"Weight loss","HPO_Feature_Type__c":"Symptom"}},{"Provided_By__c":"ORPHA:52416","HPO_Frequency__c":"Very frequent (80-99%)","Feature__r":{"HPO_Description__c":"Enlargement (swelling) of a lymph node.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0002716","HPO_Synonym__c":"Lymph node hyperplasia; Swollen lymph nodes","HPO_Name__c":"Lymphadenopathy","Feature_System__c":"Cardiovascular System; Immune System","HPO_Feature_Type__c":"Symptom"}},{"Provided_By__c":"ORPHA:52416","HPO_Frequency__c":"Frequent (30-79%)","Feature__r":{"HPO_Description__c":"A subjective feeling of tiredness characterized by a lack of energy and motivation.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0012378","HPO_Synonym__c":"Fatigue; Tired; Tiredness","HPO_Name__c":"Fatigue","HPO_Feature_Type__c":"Symptom"}},{"Provided_By__c":"ORPHA:52416","HPO_Frequency__c":"Frequent (30-79%)","Feature__r":{"HPO_Description__c":"Abnormal increased size of the spleen.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0001744","HPO_Synonym__c":"Increased spleen size; Large spleen","HPO_Name__c":"Splenomegaly","Feature_System__c":"Cardiovascular System; Immune System; Digestive System","HPO_Feature_Type__c":"Symptom"}},{"Provided_By__c":"ORPHA:52416","HPO_Frequency__c":"Frequent (30-79%)","Feature__r":{"HPO_Description__c":"An anomaly of the form or number of cells in the bone marrow.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0005561","HPO_Synonym__c":"Abnormality of bone marrow cell morphology; Anomaly of the bone marrow cells","HPO_Name__c":"Abnormal bone marrow cell morphology","Feature_System__c":"Blood and Blood-Forming Tissue","HPO_Feature_Type__c":"Symptom"}},{"Provided_By__c":"ORPHA:52416","HPO_Frequency__c":"Very frequent (80-99%)","Feature__r":{"HPO_Description__c":"A type of lymphoma that originates in B-cells.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0012191","HPO_Synonym__c":"B cell lymphoma","HPO_Name__c":"B-cell lymphoma","Feature_System__c":"Blood and Blood-Forming Tissue","HPO_Feature_Type__c":"Symptom"}},{"Provided_By__c":"ORPHA:52416","HPO_Frequency__c":"Frequent (30-79%)","Feature__r":{"HPO_Description__c":"Body temperature elevated above the normal range.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0001945","HPO_Synonym__c":"Fever; Hyperthermia; Pyrexia","HPO_Name__c":"Fever","HPO_Feature_Type__c":"Symptom"}},{"Provided_By__c":"ORPHA:52416","HPO_Frequency__c":"Occasional (5-29%)","Feature__r":{"HPO_Description__c":"An abnormality of the gastrointestinal tract.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0011024","HPO_Synonym__c":"Abnormality of the gastrointestinal tract; Abnormality of the GI tract","HPO_Name__c":"Abnormality of the gastrointestinal tract","Feature_System__c":"Digestive System","HPO_Feature_Type__c":"Symptom"}},{"Provided_By__c":"ORPHA:52416","HPO_Frequency__c":"Frequent (30-79%)","Feature__r":{"HPO_Description__c":"Lack of desire to eat (loss of appetite).","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0002039","HPO_Synonym__c":"Anorexia; Deliberately not eating; Obsessive dieting; Refusing to eat","HPO_Name__c":"Anorexia","Feature_System__c":"Digestive System","HPO_Feature_Type__c":"Symptom"}}],"tags":{"Specialist":["Cancer - Oncologist","Hematology"],"Disease Category":["Cancer","Hematology"]},"synonyms":["classical mantle cell lymphoma"," lcm"," mantle zone lymphoma"," mcl"]}