{"orpha":279897,"name":"Primary oculocerebral lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Primary oculocerebral non-Hodgkin lymphoma"],"definition":"Primary oculocerebral lymphoma is a rare, primary, organ-specific, extranodal non-Hodgkin's lymphoma (typically diffuse large B-cell lymphoma), simultaneously affecting the intraocular compartments (retina, vitreous, optic nerve, uvea and others) and the central nervous system (commonly the cerebellum, spinal cord or pia mater). The presenting symptoms vary depending on the localization of the tumor and may include vitreous floaters or blurred vision, raised intracranial pressure (headache, vomiting, papilledema) and/or focal neurological deficits.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-279897/","orphanet_url":"https://www.orpha.net/en/disease/detail/279897","names":{"fr":{"name":"Lymphome oculo-cérébral primitif","synonyms":["LOCP","Lymphome oculo-cérébral primitif non hodgkinien"],"definition":"Le lymphome oculo-cérébral primitif est un lymphome non-hodgkinien primaire rare, extra-ganglionnaire et spécifique de l'organe (habituellement, un lymphome diffus à grandes cellules B). Il affecte à la fois les compartiments intraoculaires (rétine, vitré, nerf optique, uvée et autres) et le système nerveux central (surtout le cervelet, la moelle épinière ou la pie-mère). Les signes de présentation varient selon la localisation de la tumeur et peuvent inclure des corps flottants du vitré ou une vision floue, une augmentation de la pression intracrânienne (céphalées, vomissements, oedème papillaire) et/ou des troubles neurologiques focaux."},"de":{"name":"Lymphom, okulozerebrales primäres","synonyms":["Non-Hodgkin-Lymphom, okulozerebrales primäres","Okulozerebrales NHL"],"definition":"Das primäre okulozerebrale Lymphom ist ein seltenes, primäres, organspezifisches, extranodales Non-Hodgkin-Lymphom (typischerweise diffuses großzelliges B-Zell-Lymphom), das gleichzeitig die intraokularen Kompartimente (Netzhaut, Glaskörper, Sehnerv, Uvea und andere) und das zentrale Nervensystem (häufig das Kleinhirn, Rückenmark oder die Pia mater) befällt. Die präsentierenden Symptome variieren je nach Lokalisation des Tumors und können Glaskörpertrübungen oder verschwommenes Sehen, erhöhten intrakraniellen Druck (Kopfschmerzen, Erbrechen, Papillenödem) und/oder fokale neurologische Defizite umfassen."},"es":{"name":"Linfoma oculocerebral primario","synonyms":["Linfoma no Hodgkin oculocerebral primario"],"definition":"El linfoma oculocerebral primario es un linfoma no-Hodgkin (típicamente linfoma difuso de células grandes B) extranodal, primario, órgano-específico, poco frecuente, que afecta simultáneamente a los compartimentos intraoculares (retina, cámara vítrea, nervio óptico, úvea y otros) y al sistema nervioso central (comúnmente cerebelo, médula espinal o piamadre). Los síntomas de presentación varían dependiendo de la localización del tumor y pueden incluir opacidades vítreas (moscas volantes o cuerpos flotantes) o visión borrosa, hipertensión intracraneal (dolor de cabeza, vómitos, papiledema) y/o deficiencias neurológicas focales."},"it":{"name":"Linfoma oculocerebrale primitivo","synonyms":["Linfoma non-Hodgkin oculocerebrale primitivo"],"definition":"Il linfoma oculocerebrale primitivo è un linfoma non-Hodgkin raro primitivo extranodale organo-specifico (di solito si tratta di un linfoma diffuso a grandi cellule B), che interessa contemporaneamente i compartimenti intraoculari (retina, vitreo, nervo ottico, uvea e altri) e il sistema nervoso centrale (di solito il cervelletto, il midollo spinale o la pia madre). I sintomi d'esordio variano a seconda della localizzazione del tumore e comprendono le miodesopsie o l'annebbiamento della vista, l'aumento della pressione intracranica (cefalea, vomito, papilledema) e/o i deficit neurologici focali."},"nl":{"name":"Primair oculocerebraal lymfoom","synonyms":["Primair oculocerebraal non-Hodgkin-lymfoom"],"definition":"Primair oculocerebraal lymfoom is een zeldzaam, primair, orgaan-specifiek, extranodaal non-Hodgkinlymfoom (doorgaans diffuus grootcellig B-lymfoom) dat simultaan de intra-oculaire compartimenten (netvlies, glasachtig lichaam, gezichtszenuw, uvea (druifvlies) en andere) en het centrale zenuwstelsel (doorgaans het cerebellum, het ruggenmerg of pia mater (zacht hersenvlies)) treft. De symptomen variëren afhankelijk van de lokalisatie van de tumor en omvatten mogelijk onder meer glasvochttroebeling of troebel zicht, verhoogde intracraniële druk (hoofdpijn, braken, papiloedeem) en/of focale neurologische tekorten."},"pt":{"name":"Linfoma oculocerebral primário","synonyms":["Linfoma não-Hodgkin oculocerebral primário"]},"pl":{"name":"Pierwotny chłoniak oczno-mózgowy","synonyms":["Primary oculocerebral non-Hodgkin lymphoma"]},"cs":{"name":"Primární okulocerebrální lymfom","synonyms":["Primární okulocerebrální non-Hodgkinský lymfom"]},"uk":{"name":"Первинна окулоцеребральна лімфома","synonyms":["Первинна окулоцеребральна неходжкінська лімфома"],"definition":"Primary oculocerebral lymphoma is a rare, primary, organ-specific, extranodal non-Hodgkin's lymphoma (typically diffuse large B-cell lymphoma), simultaneously affecting the intraocular compartments (retina, vitreous, optic nerve, uvea and others) and the central nervous system (commonly the cerebellum, spinal cord or pia mater). The presenting symptoms vary depending on the localization of the tumor and may include vitreous floaters or blurred vision, raised intracranial pressure (headache, vomiting, papilledema) and/or focal neurological deficits."},"zh":{"name":"原发性眼脑淋巴瘤","synonyms":["原发性眼脑非霍奇金淋巴瘤"],"definition":"原发性眼脑淋巴瘤是一种罕见的原发性、器官特异性，淋巴结外非霍奇金淋巴瘤（尤其是弥漫性大B细胞淋巴瘤），可同时影响眼内区（视网膜，玻璃体，视神经，葡萄膜等）和中枢神经系统（通常是小脑，脊髓或软脑膜）。症状因肿瘤部位而异，包括玻璃体漂浮物或视力模糊，颅内压升高（头痛，呕吐，乳头水肿）和/或局灶性神经功能缺损。"},"tr":{"name":"Primer oküloserebral lenfoma","synonyms":["Primer oküloserebral Hodgkin olmayan lenfoma"]}},"xrefs":[{"source":"ICD-10","code":"C85.7","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2B33.5","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"446504143"},{"source":"MONDO","code":"0017205","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5679779","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":279911,"name":"Primary organ-specific lymphoma","classifications":[194,202,233]},{"orpha":101950,"name":"Rare eye tumor","classifications":[189,202]},{"orpha":98062,"name":"Rare nervous system tumor","classifications":[181,202]}],"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":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}