{"orpha":86893,"name":"Nodular lymphocyte predominant Hodgkin lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["NLPHL"],"definition":"Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a rare subtype of Hodgkin lymphoma (HL) characterized histologically by malignant lymphocyte predominant (LP) cells and the absence of typical Hodgkin and Reed-Sternberg (HRS) cells.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-86893/","orphanet_url":"https://www.orpha.net/en/disease/detail/86893","names":{"fr":{"name":"Lymphome de Hodgkin nodulaire à prédominance lymphocytaire","synonyms":["LHNPL","NLPHL","Paragranulome nodulaire hodgkinien de Poppema et Lennert"],"definition":"Le lymphome de Hodgkin nodulaire à prédominance lymphocytaire (LHNPL) est un sous-type rare de lymphome de Hodgkin (LH) caractérisé sur le plan histologique par des cellules à prédominance lymphocytaire (PL) malignes et l'absence de cellules typiques de Hodgkin et de Reed-Sternberg (HRS)."},"de":{"name":"Hodgkin-Lymphom, lymphozytenprädominantes noduläres","synonyms":["NLPHL"],"definition":"Das lymphozytenprädominante noduläre Hodgkin-Lymphom (NLPHL) ist ein seltener Subtyp des Hodgkin-Lymphoms (HL; siehe dort) und histologisch gekennzeichnet durch maligne lymphpzytenprädominante (LP) Zellen und das Fehlen von typischen Hodgkin- und Reed-Sternberg (HRS)-Zellen."},"es":{"name":"Linfoma de Hodgkin con predominio de linfocitos nodulares","synonyms":["NLPHL"],"definition":"El linfoma de Hodgkin nodular de predominio linfocítico (NLPHL) es un subtipo poco frecuente del linfoma de Hodgkin (HL; consulte este término) que se caracteriza histológicamente por el predominio de linfocítos (PL) malignos, con ausencia de las células típicas de Hodgkin y Reed-Sternberg (HRS)."},"it":{"name":"Linfoma nodulare di Hodgkin a predominanza linfocitaria","synonyms":["NLPHL"],"definition":"Il linfoma di Hodgkin nodulare a predominanza linfocitaria (NLPHL) è un sottotipo raro di linfoma di Hodgkin (HL; si veda questo termine), caratterizzato istologicamente da cellule maligne a predominanza linfocitaria (LP) e dall'assenza delle caratteristiche cellule di Hodgkin e di Reed-Sternberg (HRS)."},"nl":{"name":"Nodulair lymfocyt-predominant Hodgkin-lymfoom","synonyms":["NLPHL"],"definition":"Nodulair lymfocyt-predominant Hodgkin-lymfoom (NLPHL) is een zeldzaam subtype van hodgkin-lymfoom (HL), histologisch gekenmerkt door maligne lymfocyt-predominante (LP) cellen en de afwezigheid van typische Hodgkin- en Reed-Sternberg (HRS)-cellen."},"pt":{"name":"Linfoma de Hodgkin nodular com predomínio de linfócitos"},"pl":{"name":"Chłoniak Hodgkina guzkowy z przewagą limfocytów","synonyms":["NLPHL"]},"cs":{"name":"Nodulární Hodgkinův lymfom s převahou lymfocytů","synonyms":["NLPHL"]},"uk":{"name":"Вузлова лімфоцитарна лімфома Ходжкіна з переважанням лімфоцитів","synonyms":["NLPHL"],"definition":"Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a rare subtype of Hodgkin lymphoma (HL; see this term) characterized histologically by malignant lymphocyte predominant (LP) cells and the absence of typical Hodgkin and Reed-Sternberg (HRS) cells."},"zh":{"name":"结节性淋巴细胞为主型霍奇金淋巴瘤","synonyms":["NLPHL"]},"tr":{"name":"Nodüler lenfosit dominant Hodgkin lenfoma","synonyms":["NLPHL"]}},"xrefs":[{"source":"ICD-10","code":"C81.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2B30.0","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"331115338"},{"source":"MONDO","code":"0044778","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1334968","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10080201","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":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Unknown"],"onset":["All ages"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.12,"area":"Worldwide","status":"Validated","source":"[EXPERT]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.095,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.041,"area":"Austria","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.006,"area":"Bulgaria","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.099,"area":"Czech Republic","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.037,"area":"Estonia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.097,"area":"Germany","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.074,"area":"Ireland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.038,"area":"Latvia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.008,"area":"Lithuania","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.013,"area":"Poland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.055,"area":"Portugal","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.053,"area":"Slovakia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.056,"area":"Slovenia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.092,"area":"United Kingdom","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":0.128,"area":"Belgium","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":0.285,"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":0.124,"area":"Italy","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":0.226,"area":"Norway","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":0.14,"area":"Spain","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":0.135,"area":"Switzerland","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":0.133,"area":"Netherlands","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0002721","label":"Immunodeficiency","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:0001945","label":"Fever","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0005561","label":"Abnormality of bone marrow cell morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002039","label":"Anorexia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012191","label":"B-cell lymphoma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003002","label":"Breast carcinoma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002240","label":"Hepatomegaly","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000975","label":"Hyperhidrosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000989","label":"Pruritus","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001744","label":"Splenomegaly","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Occasional (29-5%)","criteria":null}]}}