{"orpha":543,"name":"Burkitt lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Small non-cleaved cell lymphoma"],"definition":"Burkitt lymphoma is a rare form of malignant mature B-cell non-Hodgkin lymphoma.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-543/","orphanet_url":"https://www.orpha.net/en/disease/detail/543","names":{"fr":{"name":"Lymphome de Burkitt","synonyms":["Lymphome à petites cellules non clivées"],"definition":"Le lymphome de Burkitt est une forme rare de lymphome malin non-Hodgkinien à cellules B matures."},"de":{"name":"Burkitt-Lymphom"},"es":{"name":"Linfoma de Burkitt","synonyms":["Linfoma de células pequeñas no hendidas"],"definition":"Es una forma poco frecuente de linfoma no Hodgkin de células B maduras malignas."},"it":{"name":"Linfoma di Burkitt","synonyms":["Linfoma a piccole cellule non clivate"]},"nl":{"name":"Burkitt-lymfoom","synonyms":["Klein niet-gesplitst cellymfoom"],"definition":"Een zeldzame vorm van maligne non-Hodgkin-lymfoom met rijpe B-cellen."},"pt":{"name":"Linfoma de Burkitt","synonyms":["Linfoma de células pequenas e não-clivadas"]},"pl":{"name":"Chłoniak Burkitta","synonyms":["Chłoniak z małych komórek bez wpuklonego jądra komórkowego"]},"cs":{"name":"Burkittův lymfom","synonyms":["Lymfom malých buněk s neštěpeným jádrem"]},"uk":{"name":"Лімфома Беркітта","synonyms":["Невелика неклітинна лімфома"],"definition":"Burkitt lymphoma is a rare form of malignant mature B-cell non-Hodgkin lymphoma."},"zh":{"name":"伯基特淋巴瘤","synonyms":["小无裂细胞性淋巴瘤"]},"tr":{"name":"Burkitt lenfoma","synonyms":["Küçük bölünmemiş hücreli lenfoma"]}},"xrefs":[{"source":"OMIM","code":"113970","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"C83.7","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"C91.8","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"2A85.6","qualifier":"NTBT","icd_relation":"specific","validated":true,"icd11_uri":"2100138081"},{"source":"ICD-11","code":"XH4KA9","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"808906009"},{"source":"MONDO","code":"0007243","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D002051","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0006413","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"5973","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10006595","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":300846,"name":"Aggressive B-cell non-Hodgkin lymphoma","classifications":[194,202,233]},{"orpha":289644,"name":"Epstein-Barr virus-associated malignant lymphoproliferative disorder","classifications":[202]}],"children":[],"genes":[{"symbol":"MYC","name":"MYC proto-oncogene, bHLH transcription factor","gene_type":"gene with protein product","association":"Part of a fusion gene in","status":"Assessed","source":null,"hgnc":"7553","ensembl":"ENSG00000136997","omim":"190080","loci":["8q24.21"]}],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.17,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe 2003[OTHER]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-5 / 10 000","value":12.5,"area":"Africa","status":"Validated","source":"National OTHERitutes of Health[OTHER]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.4,"area":"United States","status":"Validated","source":"National Cancer OTHERitute 2001-2009[OTHER]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2017-01-03","source":null,"terms":[{"hpo":"HP:0005561","label":"Abnormality of bone marrow cell morphology","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002149","label":"Hyperuricemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0025435","label":"Increased circulating lactate dehydrogenase concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100649","label":"Neoplasm of the oral cavity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001392","label":"Abnormality of the liver","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002733","label":"Abnormality of the lymph nodes","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000137","label":"Abnormality of the ovary","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001732","label":"Abnormality of the pancreas","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001743","label":"Abnormality of the spleen","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0032218","label":"Decreased proportion of CD4-positive T cells","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002239","label":"Gastrointestinal hemorrhage","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0005214","label":"Intestinal obstruction","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002017","label":"Nausea and vomiting","frequency":"Occasional (29-5%)","criteria":null}]}}