{"orpha":86869,"name":"Lymphomatoid granulomatosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["LYG"],"definition":"Lymphomatoid granulomatosis (LYG) is a very rare Epstein-Barr virus (EBV)-driven lymphoproliferative disease most commonly occurring in adults (in the fourth to sixth decade of life) and commonly affecting the lungs (with presentations varying from small bilateral pulmonary nodules to large necrotic and sometimes cavitating lesions), skin, central nervous system, and kidneys, but only very rarely affecting the lymph nodes and spleen. The symptoms associated with LYG depend on the site of disease involvement but mainly include cough, dyspnea or chest pain (in those with pulmonary involvement) and constitutional symptoms such as weight loss and fever.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-86869/","orphanet_url":"https://www.orpha.net/en/disease/detail/86869","names":{"fr":{"name":"Granulomatose lymphomatoïde","synonyms":["LYG"],"definition":"Syndrome lymphoprolifératif très rare associé au virus Epstein-Barr (EBV), survenant le plus souvent chez l'adulte (entre la quatrième et la sixième décennie de vie) et affectant fréquemment les poumons (les signes cliniques allant de petits nodules pulmonaires bilatéraux à de grandes lésions nécrotiques et parfois cavitaires), la peau, le système nerveux central et les reins, mais n'affectant que très rarement les ganglions lymphatiques et la rate. Les symptômes associés dépendent du site atteint et comprennent principalement une toux, une dyspnée ou une douleur thoracique (dans les cas d'atteinte pulmonaire) et des symptômes constitutionnels tels qu'une perte de poids et une fièvre."},"de":{"name":"Lymphomatoide Granulomatose","synonyms":["LYG"]},"es":{"name":"Granulomatosis linfomatoide","synonyms":["LYG"],"definition":"Es una enfermedad linfoproliferativa muy poco frecuente causada por el virus de Epstein-Barr (VEB) que se presenta con mayor frecuencia en adultos (de la cuarta a la sexta década de la vida) afectando principalmente a los pulmones (con presentaciones que varían desde pequeños nódulos pulmonares bilaterales, hasta lesiones necróticas grandes y, en ocasiones, cavitadas). La enfermedad también afecta a la piel, al sistema nervioso central, a los riñones, y, de manera excepcional, a los ganglios linfáticos y al bazo. Los síntomas asociados dependen del área afectada, pero principalmente incluyen tos, disnea o dolor de pecho (en aquellos con afectación pulmonar) y síntomas constitucionales tales como pérdida de peso y fiebre."},"it":{"name":"Granulomatosi linfomatoide","synonyms":["LYG"],"definition":"La granulomatosi linfomatoide (LYG) è una malattia linfoproliferativa molto rara causata dal virus di Epstein-Barr (EBV), che colpisce di solito gli adulti (nella quarta-sesta decade di vita), interessando in genere i polmoni (i segni clinici comprendono i piccoli noduli polmonari bilaterali fino alle lesioni necrotiche di ampie dimensioni talvolta cavitarie), la cute, il sistema nervoso centrale e i reni; molto raramente interessa i linfonodi e la milza. I sintomi associati alla LYG dipendono dalla localizzazione della malattia, ma comprendono di regola la tosse, la dispnea o il dolore toracico (nei casi con interessamento polmonare) e alcuni sintomi costituzionali come la perdita di peso e la febbre."},"nl":{"name":"Lymfomatoïde granulomatose","synonyms":["LYG"],"definition":"Lymfomatoïde granulomatose (LYG) is een zeer zeldzame lymfoproliferatieve ziekte die wordt veroorzaakt door Epstein-Barr virus (EBV), die meestal voorkomt bij volwassenen (in het vierde tot zesde levensdecennium), en die doorgaans de longen (met manifestaties gaande van kleine bilaterale pulmonale noduli tot grote necrotische en soms holtevormende laesies), de huid, het centrale zenuwstelsel, en de nieren treft, maar slechts zeer uitzonderlijk de lymfeklieren en de milt. De symptomen die zijn geassocieerd met LYG hangen af van waar de ziekte toeslaat, maar omvatten voornamelijk hoest, dyspneu of pijn in de borst (in diegenen met betrokkenheid van de longen) en constitutionele symptomen zoals gewichtsverlies en koorts."},"pt":{"name":"Granulomatose linfomatóide"},"pl":{"name":"Ziarniniakowatość limfoidalna (nazwa polska jest myląca, gdyż wskazuje na zmianę łagodną co nie jest prawdą i dlatego lepiej nie tłumaczyć nazwy anglojęzycznej)","synonyms":["LYG"]},"cs":{"name":"Lymfomatoidní granulomatóza","synonyms":["LYG"]},"uk":{"name":"Лімфоматоїдний гранулематоз","synonyms":["LYG"],"definition":"Lymphomatoid granulomatosis (LYG) is a very rare Epstein-Barr virus (EBV)-driven lymphoproliferative disease most commonly occurring in adults (in the fourth to sixth decade of life) and commonly affecting the lungs (with presentations varying from small bilateral pulmonary nodules to large necrotic and sometimes cavitating lesions), skin, central nervous system, and kidneys, but only very rarely affecting the lymph nodes and spleen. The symptoms associated with LYG depend on the site of disease involvement but mainly include cough, dyspnea or chest pain (in those with pulmonary involvement) and constitutional symptoms such as weight loss and fever."},"zh":{"name":"淋巴瘤样肉芽肿","synonyms":["LYG"]},"tr":{"name":"Lenfomatoid granülomatoz","synonyms":["LYG"]}},"xrefs":[{"source":"ICD-10","code":"C83.8","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"2A81.3","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1890408959"},{"source":"MONDO","code":"0019466","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D008230","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0024307","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"6943","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10025325","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":544,"name":"Diffuse large B-cell lymphoma","classifications":[194,202,233]},{"orpha":289644,"name":"Epstein-Barr virus-associated malignant lymphoproliferative disorder","classifications":[202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}