{"orpha":391,"name":"Classic Hodgkin lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Classic Hodgkin disease"],"definition":"Classical Hodgkin lymphoma (CHL) is a B-cell lymphoma characterized histologically by the presence of large mononuclear Hodgkin cells and multinucleated Reed-Sternberg (HRS) cells.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-391/","orphanet_url":"https://www.orpha.net/en/disease/detail/391","names":{"fr":{"name":"Lymphome de Hodgkin classique","synonyms":["Maladie de Hodgkin classique"],"definition":"Le lymphome de Hodgkin classique (LHc) est un lymphome à cellules B caractérisé sur le plan histologique par la présence de cellules de Hodgkin mononucléées de grande taille et de cellules polynucléées de Reed-Sternberg (CRS)."},"de":{"name":"Hodgkin-Lymphom, klassisches","synonyms":["Hodgkin-Krankheit, klassische"],"definition":"Das klassische Hodgkin-Lymphom (CHL) ist ein B-Zell-Lymphom und histologisch durch das Vorhandensein großer mononukleärer Hodgkin-Zellen und mehrkerniger Reed-Sternberg (HRS)-Zellen gekennzeichnet."},"es":{"name":"Linfoma de Hodgkin clásico","synonyms":["Enfermedad de Hodgkin clásica"],"definition":"El linfoma de Hodgkin clásico (CHL) es un linfoma de células B que se caracteriza histológicamente por la presencia de células de Hodgkin mononucleares grandes y células de Reed-Sternberg (HRS) multinucleadas."},"it":{"name":"Linfoma di Hodgkin classico","synonyms":["Malattia di Hodgkin classica"],"definition":"Il linfoma di Hodgkin classico (CHL) è un linfoma a cellule B, caratterizzato istologicamente da grandi cellule mononucleate di Hodgkin e cellule multinucleate di Reed-Sternberg (HRS)."},"nl":{"name":"Klassiek Hodgkin-lymfoom","synonyms":["Klassieke ziekte van Hodgkin"],"definition":"Een B-cellymfoom, histologisch gekenmerkt door de aanwezigheid van grote mononucleaire Hodgkin-cellen en multinucleaire Reed-Sternberg (HRS)-cellen."},"pt":{"name":"Doença de Hodgkin","synonyms":["Linfoma de Hodgkin"]},"pl":{"name":"Klasyczny chłoniak Hodgkina","synonyms":["Klasyczna choroba Hodgkina"]},"cs":{"name":"Klasický Hodgkinův lymfom","synonyms":["Klasická Hodgkinova choroba"]},"uk":{"name":"Класична лімфома Ходжкіна","synonyms":["Класична хвороба Ходжкіна"],"definition":"Classical Hodgkin lymphoma (CHL) is a B-cell lymphoma characterized histologically by the presence of large mononuclear Hodgkin cells and multinucleated Reed-Sternberg (HRS) cells."},"zh":{"name":"经典霍奇金淋巴瘤","synonyms":["典型霍奇金病"]},"tr":{"name":"Klasik Hodgkin lenfoma","synonyms":["Klasik Hodgkin hastalığı"]}},"xrefs":[{"source":"OMIM","code":"236000","qualifier":"E","icd_relation":null,"validated":true},{"source":"OMIM","code":"300221","qualifier":"BTNT","icd_relation":null,"validated":true},{"source":"OMIM","code":"400021","qualifier":"BTNT","icd_relation":null,"validated":true},{"source":"ICD-10","code":"C81.0","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"C81.1","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"C81.2","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"C81.3","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"C81.4","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"C81.7","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"C81.9","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"2B30.1","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1616050398"},{"source":"MONDO","code":"0009348","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1333064","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":98293,"name":"Hodgkin lymphoma","classifications":[194,202,233]}],"children":[{"orpha":98846,"name":"Classic Hodgkin lymphoma, lymphocyte-depleted type","type":"Histopathological subtype"},{"orpha":98845,"name":"Classic Hodgkin lymphoma, lymphocyte-rich type","type":"Histopathological subtype"},{"orpha":98844,"name":"Classic Hodgkin lymphoma, mixed cellularity type","type":"Histopathological subtype"},{"orpha":98843,"name":"Classic Hodgkin lymphoma, nodular sclerosis type","type":"Histopathological subtype"}],"genes":[],"genes_via_subtypes":[{"symbol":"KLHDC8B","name":"kelch domain containing 8B","gene_type":"gene with protein product","association":"Candidate gene tested in","status":"Not yet assessed","source":"19706467[PMID]","hgnc":"28557","ensembl":"ENSG00000185909","omim":"613169","loci":["3p21.31"],"via":{"orpha":98843,"name":"Classic Hodgkin lymphoma, nodular sclerosis type"}}],"inheritance":["Unknown"],"onset":["All ages"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.463,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe [OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.95,"area":"France","status":"Validated","source":"OTHERitut National du Cancer 2013[OTHER]"},{"type":"Point prevalence","qualification":"Class only","class":"1-5 / 10 000","value":0.0,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe 2010[OTHER]"},{"type":"Lifetime Prevalence","qualification":"Value and class","class":"1-5 / 10 000","value":22.9,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe 2010[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.8,"area":"United States","status":"Validated","source":"National Cancer OTHERitute 2008[OTHER]_emedicine[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.25,"area":"China","status":"Not yet validated","source":"emedicine[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.25,"area":"Japan","status":"Not yet validated","source":"emedicine[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.012,"area":"Austria","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.267,"area":"Norway","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.2,"area":"Poland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.435,"area":"Portugal","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.261,"area":"Slovakia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.265,"area":"Slovenia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.617,"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":3.091,"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":2.27,"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":2.504,"area":"United Kingdom","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.443,"area":"Belgium","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.083,"area":"Bulgaria","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.923,"area":"Croatia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.418,"area":"Czech Republic","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.392,"area":"Estonia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.232,"area":"Finland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.333,"area":"Germany","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.599,"area":"Iceland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.355,"area":"Ireland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":3.361,"area":"Italy","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":1.868,"area":"Latvia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.372,"area":"Lithuania","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.701,"area":"Malta","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2024-03-06","source":"33493434[PMID]_32703953[PMID]","terms":[{"hpo":"HP:0005374","label":"Cellular immunodeficiency","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","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:0002039","label":"Anorexia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003418","label":"Back pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100749","label":"Chest pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012735","label":"Cough","frequency":"Frequent 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(29-5%)","criteria":null},{"hpo":"HP:0009830","label":"Peripheral neuropathy","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002093","label":"Respiratory insufficiency","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000988","label":"Skin rash","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001744","label":"Splenomegaly","frequency":"Occasional (29-5%)","criteria":null}]}}