{"orpha":86886,"name":"Angioimmunoblastic T-cell lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Immunoblastic lymphadenopathy","Lymphogranulomatosis X","T-cell lymphoma, AILD type","AILT"],"definition":"A rare T-cell non-Hodgkin lymphoma characterized by infiltration of lymph nodes by neoplastic cells of T follicular helper cell origin with a polymorphous inflammatory background including markedly increased follicular dendritic cells and EBV-positive B-cells, as well as prominent proliferation of high endothelial venules. The spleen, liver, skin, and bone marrow are also frequently involved. Patients typically present with generalized lymphadenopathy, hepatosplenomegaly, systemic symptoms, and polyclonal hypergammaglobulinemia. Pruritic skin rash, arthritis, pleural effusion, and ascites may also be observed. The condition is aggressive with generally poor prognosis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-86886/","orphanet_url":"https://www.orpha.net/en/disease/detail/86886","names":{"fr":{"name":"Lymphome T angioimmunoblastique","synonyms":["AILT","Lymphadénopathie immunoblastique","Lymphogranulomatose X","Lymphome T type AILD"],"definition":"Lymphome T non hodgkinien rare caractérisé par l'infiltration des ganglions lymphatiques par des cellules néoplasiques d'origine folliculaire T helper et un fond inflammatoire avec prolifération polymorphe constituée de cellules dendritiques folliculaires et de cellules B positives à l'EBV, ainsi que de veinules postcapillaires à endothélium épais. Souvent, la rate, le foie, la peau et la moelle osseuse sont également touchés. Les patients présentent généralement une lymphadénopathie généralisée, une hépatosplénomégalie, des symptômes systémiques et une hypergammaglobulinémie polyclonale. On peut également observer une éruption cutanée prurigineuse, une arthrite, un épanchement pleural et une ascite. La maladie est agressive et son pronostic est généralement réservé."},"de":{"name":"T-Zell-Lymphom, angioimmunoblastisches","synonyms":["AILT","Lymphadenopathie, immunoblastische","Lymphogranulomatose X","T-Zell-Lymphom vom AILD-Typ"],"definition":"Ein seltenes T-Zell-Non-Hodgkin-Lymphom, das durch die Infiltration von Lymphknoten durch neoplastische Zellen follikulärer T-Helferzellen mit einem polymorphen entzündlichen Hintergrund gekennzeichnet ist, zu dem auch eine deutliche Zunahme follikulärer dendritischer Zellen und EBV-positiver B-Zellen sowie eine ausgeprägte Proliferation hoher endothelialer Venolen gehören. Häufig sind auch die Milz, die Leber, die Haut und das Knochenmark betroffen. Die Patienten weisen typischerweise eine generalisierte Lymphadenopathie, Hepatosplenomegalie, systemische Symptome und eine polyklonale Hypergammaglobulinämie auf. Juckende Hautausschläge, Arthritis, Pleuraerguss und Aszites können ebenfalls beobachtet werden. Die Erkrankung ist aggressiv und hat im Allgemeinen eine schlechte Prognose."},"es":{"name":"Linfoma angioinmunoblástico de células T","synonyms":["Linfoma de células T tipo AILD"],"definition":"Es un linfoma no Hodgkin de células T poco frecuente caracterizado por la infiltración de los ganglios linfáticos por células neoplásicas originadas a partir células T helper foliculares con un perfil inflamatorio polimorfo que incluye un incremento significativo de células foliculares dendríticas y de células B positivas para VEB, así como una proliferación prominente de vénulas de endotelio alto. El bazo, el hígado, la piel y la médula ósea también se ven afectados con frecuencia. Los pacientes suelen presentar linfadenopatía generalizada, hepatoesplenomegalia, síntomas sistémicos e hipergammaglobulinemia policlonal. También se pueden observar erupciones cutáneas pruriginosas, artritis, derrame pleural y ascitis. El trastorno es agresivo y el pronóstico suele ser desfavorable."},"it":{"name":"Linfoma angioimmunoblastico a cellule T","synonyms":["AILT","Linfadenopatia immunoblastica","Linfogranulomatosi X","Linfoma a cellule T, tipo AILD"],"definition":"Si tratta di un linfoma raro non-Hodgkin a cellule T, caratterizzato dall'infiltrazione dei linfonodi da parte delle cellule neoplastiche originate dai linfociti T helper follicolari con una base infiammatoria polimorfa che comprende un marcato aumento delle cellule dendritiche follicolari e delle cellule B EBV-positive, e una marcata proliferazione delle venule con alto endotelio. Spesso la malattia interessa anche la milza, il fegato, la cute e il midollo osseo. Di solito i pazienti presentano linfoadenopatia generalizzata, epatosplenomegalia, sintomi sistemici, ipergammaglobulinemia policlonale e, talvolta, eruzioni cutanee pruriginose, artrite, versamento pleurico e ascite. La malattia è aggressiva e la prognosi in genere è sfavorevole."},"nl":{"name":"Angio-immunoblastisch T-cellymfoom","synonyms":["AILT","Immunoblastische lymfadenopathie","Lymfogranulomatose X","T-cellymfoom, AILD-type"],"definition":"Een zeldzaam non-Hodgkinlymfoom van T-cellen, gekarakteriseerd door infiltratie van lymfeklieren door neoplastische cellen afkomstig van folliculaire T-helpercellen en een inflammatoire achtergrond met polymorfe proliferatie bestaande uit folliculaire dendritische cellen en EBV-positieve B-cellen, alsook hoog-endotheliale venulen. Vaak zijn ook milt, lever, huid en beenmerg betrokken. Patiënten presenteren zich doorgaans met gegeneraliseerde lymfadenopathie, hepatosplenomegalie, systemische symptomen, en polyklonale hypergammaglobulinemie. Pruritische huiduitslag, artritis, pleura-effusie, en ascites worden mogelijk ook waargenomen. De aandoening is agressief en heeft doorgaans een slechte prognose."},"pt":{"name":"Linfoma de células T angioimunoblástico"},"pl":{"name":"Chłoniak angioimmunoblastyczny z komórek T","synonyms":["AILT","Chłoniak z komórek T, typ AILD","Limfadenopatia limfoblastyczna","Limfogranulomatoza X"]},"cs":{"name":"T-angioimunoblastický lymfom","synonyms":["AILT","Imunoblastická lymfadenopatie","Lymfogranulomatóza X","T-buněčný lymfom, AILD typ"]},"uk":{"name":"Ангіоімунобластна Т-клітинна лімфома","synonyms":["AILT","Імунобластна лімфаденопатія","Лімфогранулематоз X","Т-клітинна лімфома, тип AILD"],"definition":"A rare T-cell non-Hodgkin lymphoma characterized by infiltration of lymph nodes by neoplastic cells of T follicular helper cell origin with a polymorphous inflammatory background including markedly increased follicular dendritic cells and EBV-positive B-cells, as well as prominent proliferation of high endothelial venules. The spleen, liver, skin, and bone marrow are also frequently involved. Patients typically present with generalized lymphadenopathy, hepatosplenomegaly, systemic symptoms, and polyclonal hypergammaglobulinemia. Pruritic skin rash, arthritis, pleural effusion, and ascites may also be observed. The condition is aggressive with generally poor prognosis."},"zh":{"name":"血管免疫母细胞性T细胞淋巴瘤","synonyms":["免疫母细胞性淋巴结病"]},"tr":{"name":"Anjiyoimmünoblastik T-hücreli lenfoma","synonyms":["AILT","Lenfogranülomatoz X","T hücreli lenfoma, AILD tipi","İmmünoblastik lenfadenopati"]}},"xrefs":[{"source":"ICD-10","code":"C86.5","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"2A90.9","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1254954229"},{"source":"MONDO","code":"0004977","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0020981","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"11973","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10002449","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":171918,"name":"T-cell non-Hodgkin lymphoma","classifications":[194,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.05,"area":"United States","status":"Validated","source":"16150940[PMID]"}],"phenotypes":null}