{"orpha":319667,"name":"Primary lymphoma of the conjunctiva","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Primary lymphoid conjunctival tumor"],"definition":"Primary lymphoma of the conjunctiva is an extremely rare clonal lymphoid proliferation of the ocular surface, with an indolent course. Clinically it presents with treatment-resistant conjunctivitis, ptosis, excessive tear production or as a painless, salmon-pink, ''fleshy'' patch, with a smooth or multinodular surface, on the bulbar conjunctiva. Histologically it is usually B-cell Non-Hodgkin lymphoma (most often extranodal marginal zone B-cell lymphoma, followed by follicular and diffuse large B-cell lymphoma), with conjunctival T-cell Non-Hodgkin lymphoma being very rare.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-319667/","orphanet_url":"https://www.orpha.net/en/disease/detail/319667","names":{"fr":{"name":"Lymphome primitif de la conjonctive","definition":"Une prolifération lymphoïde clonale de la surface oculaire, extrêmement rare et d'évolution indolente. Sur le plan clinique, elle se manifeste par une conjonctivite résistante au traitement, un ptosis, un larmoiement excessif, ou par un patch charnu, indolore, de coloration rose saumon, à la surface rugueuse ou multinodulaire, situé au niveau de la conjonctive bulbaire. Sur le plan histologique, il se manifeste habituellement sous la forme d'un lymphome B non hodgkinien (le plus souvent, un lymphome B extra-ganglionnaire de la zone marginale, suivi par un lymphome B diffus et folliculaire à grandes cellules). Le lymphome T non hodgkinien du tissu conjonctif est très rare."},"de":{"name":"Bindehaut-Lymphom, primäres","synonyms":["Primärer lymphoider Tumor der Bindehaut"],"definition":"Das primäre Lymphom der Bindehaut ist eine extrem seltene klonale lymphoide Proliferation der Augenoberfläche mit einem indolenten Verlauf. Klinisch präsentiert es sich mit behandlungsresistenter Bindehautentzündung, Ptosis, übermäßiger Tränenproduktion oder als schmerzloser, lachsfarbener, ''fleischiger'' Fleck mit glatter oder multinodulärer Oberfläche auf der bulbären Bindehaut. Histologisch handelt es sich in der Regel um ein B-Zell-Non-Hodgkin-Lymphom (am häufigsten ein extranodales Marginalzonen-B-Zell-Lymphom, gefolgt von einem follikulären und diffusen großzelligen B-Zell-Lymphom), wobei ein konjunktivales T-Zell-Non-Hodgkin-Lymphom sehr selten ist."},"es":{"name":"Linfoma primario de la conjuntiva","synonyms":["Tumor linfoide primario de la conjuntiva"],"definition":"El linfoma primario de la conjuntiva es una proliferación linfoide clonal de la superficie ocular extremadamente rara, con un curso clínico indolente. Clínicamente se presenta con conjuntivitis resistente al tratamiento, ptosis, producción excesiva de lágrimas o a modo de parches 'carnosos' indoloros, de color rosa salmón, con una superficie lisa o multinodular, en la conjuntiva bulbar. Desde el punto de vista histológico, por lo general, se trata de un linfoma no Hodgkin de células B (con mayor frecuencia linfoma de células B de zona marginal extranodal seguido de linfoma difuso de células B grandes y linfoma folicular), siendo muy infrecuente el linfoma conjuntival de células T no Hodgkin."},"it":{"name":"Linfoma primitivo della congiuntiva","synonyms":["Tumore congiuntivale linfoide primitivo"],"definition":"Il linfoma primitivo della congiuntiva è una proliferazione linfoide clonale molto rara della superficie oculare, caratterizzata da un decorso silente. I segni clinici comprendono la congiuntivite resistente al trattamento, la ptosi e l'eccessiva produzione di lacrime; la malattia può inoltre esordire sotto forma di macchia ''carnosa'' indolore di colore rosa salmone, oppure di superficie liscia o multinodulare sulla congiuntiva bulbare. Di solito, le caratteristiche istologiche sono quelle del linfoma non-Hodgkin a cellule B (perlopiù linfoma a cellule B della zona marginale extranodale, seguito dal linfoma follicolare e diffuso a grandi cellule B), mentre è molto raro il linfoma non-Hodgkin a cellule T congiuntivale."},"nl":{"name":"Primair lymfoom van conjunctiva","synonyms":["Primair lymfoom van bindvlies","Primaire lymfoïde conjunctivale tumor"],"definition":"Primair lymfoom van het bindvlies is een uiterst zeldzame klonale lymfoïde proliferatie van het oogoppervlak, met een traag verloop. Klinisch manifesteert de ziekte zich met behandelingsresistente bindvliesontsteking, ptose, overmatige traanproductie of als een pijnloze, zalmroze, vlezige vlek met een glad of multinodulair oppervlak, op de conjunctiva bulbi. Histologisch is het meestal een B-cel non-Hodgkinlymfoom (meest frequent een extranodale marginale zone B-cellymfoom, gevolgd door folliculair en diffuus grootcellig B-cellymfoom), terwijl T-cel non-Hodgkinlymfoom van het bindvlies zeer zeldzaam is."},"pt":{"name":"Linfoma primário da conjuntiva"},"pl":{"name":"Pierwotny chłoniak spojówki","synonyms":["Pierwotny guz limfoidalny rogówki"]},"cs":{"name":"Primární lymfom spojivky","synonyms":["Primární lymfoidní nádor spojivky"]},"uk":{"name":"Первинна лімфома конюнктиви","synonyms":["Первинна лімфоїдна пухлина конюнктиви"],"definition":"Primary lymphoma of the conjunctiva is an extremely rare clonal lymphoid proliferation of the ocular surface, with an indolent course. Clinically it presents with treatment-resistant conjunctivitis, ptosis, excessive tear production or as a painless, salmon-pink, ''fleshy'' patch, with a smooth or multinodular surface, on the bulbar conjunctiva. Histologically it is usually B-cell Non-Hodgkin lymphoma (most often extranodal marginal zone B-cell lymphoma, followed by follicular and diffuse large B-cell lymphoma), with conjunctival T-cell Non-Hodgkin lymphoma being very rare."},"zh":{"name":"原发性结膜淋巴瘤","synonyms":["Primary lymphoid conjunctival tumor"],"definition":"结膜原发性淋巴瘤是一种极其罕见的眼表克隆性淋巴增生，无痛性病程。临床上表现为难治性结膜炎、上睑下垂、泪液分泌过多，或在球结膜上呈无痛的橙红色“肉质”斑块，表面光滑或多结节。组织学上通常是B细胞非霍奇金淋巴瘤(最常见的是结外边缘区B细胞淋巴瘤，其次是滤泡性和弥漫性大B细胞淋巴瘤)，结膜T细胞非霍奇金淋巴瘤非常罕见。"},"tr":{"name":"Konjonktivanın primer lenfoması","synonyms":["Primer lenfoid konjonktival tümör"]}},"xrefs":[{"source":"ICD-10","code":"C85.7","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2B33.5","qualifier":"NTBT","icd_relation":"attributed","validated":true,"icd11_uri":"null"},{"source":"MONDO","code":"0017907","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4706485","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97966,"name":"Rare ophthalmic disorder"},"parents":[{"orpha":279911,"name":"Primary organ-specific lymphoma","classifications":[194,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}