{"orpha":545,"name":"Follicular lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Follicular lymphoma is a form of non-Hodgkin lymphoma characterized by a proliferation of B cells whose nodular structure of follicular architecture is preserved.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-545/","orphanet_url":"https://www.orpha.net/en/disease/detail/545","names":{"fr":{"name":"Lymphome folliculaire","definition":"Le lymphome folliculaire est une forme de lymphome non-Hodgkinien caractérisé par une prolifération de cellules B dont la structure nodulaire à architecture folliculaire est conservée."},"de":{"name":"Lymphom, follikuläres","definition":"Das Follikuläre Lymphom ist eine Form des Non-Hodgkin-Lymphoms (s. dort) mit Proliferation von B-Zellen, deren nodulär strukturierte Follikelarchitektur erhalten bleibt."},"es":{"name":"Linfoma folicular","definition":"El linfoma folicular es una forma de linfoma no Hodgkin (véase este término) que se caracteriza por una proliferación de células B cuya estructura nodular de la arquitectura folicular está conservada."},"it":{"name":"Linfoma follicolare"},"nl":{"name":"Folliculair lymfoom","definition":"Een vorm van non-Hodgkin-lymfoom, gekenmerkt door proliferatie van B-cellen waarvan de nodulaire structuur van de folliculaire architectuur bewaard is gebleven."},"pt":{"name":"Linfoma folicular"},"pl":{"name":"Chłoniak grudkowy"},"cs":{"name":"Folikulární lymfom"},"uk":{"name":"Фолікулярна лімфома","definition":"Follicular lymphoma is a form of non-Hodgkin lymphoma (see this term) characterized by a proliferation of B cells whose nodular structure of follicular architecture is preserved."},"zh":{"name":"滤泡型淋巴瘤"},"tr":{"name":"Foliküler lenfoma"}},"xrefs":[{"source":"OMIM","code":"613024","qualifier":"BTNT","icd_relation":null,"validated":true},{"source":"ICD-10","code":"C82.0","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"C82.1","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"C82.2","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"C82.3","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"C82.4","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"C82.5","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"C82.6","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"C82.7","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"C82.9","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"2A80","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"797822185"},{"source":"ICD-11","code":"2A80.0","qualifier":"BTNT","icd_relation":"specific","validated":true,"icd11_uri":"305908598"},{"source":"ICD-11","code":"2A80.1","qualifier":"BTNT","icd_relation":"specific","validated":true,"icd11_uri":"2003830496"},{"source":"ICD-11","code":"2A80.2","qualifier":"BTNT","icd_relation":"specific","validated":true,"icd11_uri":"64837151"},{"source":"ICD-11","code":"2A80.4","qualifier":"BTNT","icd_relation":"specific","validated":true,"icd11_uri":"293282687"},{"source":"ICD-11","code":"2A80.5","qualifier":"BTNT","icd_relation":"specific","validated":true,"icd11_uri":"871952666"},{"source":"ICD-11","code":"2A80.6","qualifier":"BTNT","icd_relation":"specific","validated":true,"icd11_uri":"1792850491"},{"source":"ICD-11","code":"2A80.Y","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018906","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D008224","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0024301","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"2356","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10085128","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":300842,"name":"Indolent B-cell non-Hodgkin lymphoma","classifications":[194,202,233]}],"children":[],"genes":[{"symbol":"HLA-DRB1","name":"major histocompatibility complex, class II, DR beta 1","gene_type":"gene with protein product","association":"Major susceptibility factor in","status":"Assessed","source":"23791106[PMID]","hgnc":"4948","ensembl":"ENSG00000196126","omim":"142857","loci":["6p21.32"]},{"symbol":"BCL2","name":"BCL2 apoptosis regulator","gene_type":"gene with protein product","association":"Part of a fusion gene in","status":"Assessed","source":"23953419[PMID]_22684552[PMID]","hgnc":"990","ensembl":"ENSG00000171791","omim":"151430","loci":["18q21.33"]},{"symbol":"BCL6","name":"BCL6 transcription repressor","gene_type":"gene with protein product","association":"Part of a fusion gene in","status":"Assessed","source":"22968395[PMID]","hgnc":"1001","ensembl":"ENSG00000113916","omim":"109565","loci":["3q27.3"]},{"symbol":"IGH","name":"immunoglobulin heavy locus","gene_type":"gene with protein product","association":"Part of a fusion gene in","status":"Assessed","source":"23953419[PMID]","hgnc":"5477","ensembl":null,"omim":"146910","loci":["14q32.33"]}],"genes_via_subtypes":[],"inheritance":["Multigenic/multifactorial","Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-5 / 10 000","value":37.0,"area":"Europe","status":"Validated","source":"European Medicines Agency 2018[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":3.95,"area":"France","status":"Validated","source":"OTHERitut National du Cancer 2013[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.192,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.348,"area":"Bulgaria","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.72,"area":"Estonia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.579,"area":"Finland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.363,"area":"Latvia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and 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of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.375,"area":"Spain","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.916,"area":"Switzerland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.977,"area":"Netherlands","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.766,"area":"United Kingdom","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2024-01-15","source":"30855794[PMID]_31814159[PMID]","terms":[{"hpo":"HP:0001945","label":"Fever","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002716","label":"Lymphadenopathy","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002665","label":"Lymphoma","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0100721","label":"Mediastinal lymphadenopathy","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030166","label":"Night sweats","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001744","label":"Splenomegaly","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002585","label":"Abnormality of the peritoneum","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001541","label":"Ascites","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003072","label":"Hypercalcemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025435","label":"Increased circulating lactate dehydrogenase concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001004","label":"Lymphedema","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001287","label":"Meningitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002202","label":"Pleural effusion","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0200036","label":"Skin nodule","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0033823","label":"Mediastinal mass","frequency":"Very rare (<4-1%)","criteria":null}]}}