{"orpha":86880,"name":"Enteropathy-associated T-cell lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["EATL","ETTL","Enteropathy-associated T-cell lymphoma type 1","Enteropathy-type T-cell lymphoma","Intestinal T-cell lymphoma"],"definition":"A rare T-cell non-Hodgkin lymphoma characterized by a neoplasm of intraepithelial T-cells mostly occurring in the jejunum or ileum in patients with celiac disease. The lesion may be multifocal and form ulcerating nodules, plaques, strictures, or an exophytic mass. The mesentery and mesenteric lymph nodes are commonly involved. Patients typically present with abdominal pain, malabsorption or diarrhea, anorexia, weight loss, fatigue, nausea, vomiting, and sometimes intestinal perforation or hemorrhage. Prognosis is generally poor.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-86880/","orphanet_url":"https://www.orpha.net/en/disease/detail/86880","names":{"fr":{"name":"Lymphome T associé à une entéropathie","synonyms":["EATL","ETTL","Lymphome T associé à une entéropathie de type 1","Lymphome T intestinal","Lymphome T type entéropathie"],"definition":"Lymphome T non hodgkinien caractérisé par un néoplasme à cellules T intra-épithélial survenant principalement dans le jéjunum ou l'iléon chez les patients atteints de la maladie coeliaque. La lésion peut être multifocale et former des nodules ulcérants, des plaques, des stries ou une masse exophytique. Le mésentère et les ganglions lymphatiques mésentériques sont fréquemment touchés. Les patients manifestent généralement des douleurs abdominales, une malabsorption ou une diarrhée, une anorexie, une perte de poids, une fatigue, des nausées, des vomissements et parfois une perforation ou une hémorragie intestinale. Le pronostic est généralement réservé."},"de":{"name":"Enteropathie-assoziiertes T-Zell-Lymphom","synonyms":["EATL","ETTL","Enteropathie-assoziiertes T-Zell-Lymphom Typ 1","T-Zell-Lymphom vom Enteropathie-Typ","T-Zell-Lymphom, intestinales"],"definition":"Ein T-Zell-Non-Hodgkin-Lymphom, bei dem es sich um eine Neoplasie intraepithelialer T-Zellen handelt, die meist im Jejunum oder Ileum bei Patienten mit Zöliakie auftritt. Die Läsion kann multifokal sein und ulzerierende Knötchen, Plaques, Strikturen oder eine exophytische Gewebeverdichtung bilden. Das Mesenterium und die Mesenteriallymphknoten sind in der Regel betroffen. Die Patienten zeigen typischerweise Bauchschmerzen, Malabsorption oder Durchfall, Anorexie, Gewichtsverlust, Müdigkeit, Übelkeit, Erbrechen und manchmal Darmperforation oder Blutungen. Die Prognose ist im Allgemeinen schlecht."},"es":{"name":"Linfoma de células T asociado a enteropatía","synonyms":["EATL","ETTL","Linfoma de células T asociado a enteropatía tipo 1","Linfoma de células T tipo enteroptía","Linfoma intestinal de células T"],"definition":"Es un linfoma no Hodgkin de células T poco frecuente caracterizado por una neoplasia de células T intraepiteliales que aparece principalmente en el yeyuno o el íleon en pacientes con enfermedad celíaca. La lesión puede ser multifocal y formar nódulos ulcerativos, placas, estenosis o una masa exofítica. El mesenterio y los ganglios linfáticos mesentéricos suelen estar afectados. Los pacientes se presentan típicamente con dolor abdominal, malabsorción o diarrea, anorexia, pérdida de peso, cansancio, náuseas, vómitos y, en ocasiones, perforación o hemorragia intestinal. Por lo general, el pronóstico es desfavorable."},"it":{"name":"Linfoma a cellule T associato a enteropatia","synonyms":["EATL","ETTL","Linfoma a cellule T-enteropatia","Linfoma intestinale a cellule T","Linfoma tipo 1 a cellule T associato a enteropatia"],"definition":"Si tratta di un linfoma raro non Hodgkin a cellule T, caratterizzato da una neoplasia delle cellule T intraepiteliali, localizzato prevalentemente nel digiuno o nell'ileo dei pazienti con celiachia. La lesione può essere multifocale e associarsi a noduli ulcerati, placche, stenosi o ad una massa esofitica. È comune l'interessamento del mesentere e dei linfonodi mesenterici. Di solito i pazienti presentano dolore addominale, malassorbimento o diarrea, anoressia, perdita di peso, affaticamento, nausea, vomito e, talvolta, perforazione o emorragia intestinale. In genere la prognosi è sfavorevole."},"nl":{"name":"Enteropathie-geassocieerd T-cellymfoom","synonyms":["EATL","ETTL","Enteropathie-geassocieerd T-cellymfoom type 1","Enteropathie-type T-cellymfoom","Intestinaal T-cellymfoom"],"definition":"Een zeldzaam T-cel non-Hodgkinlymfoom, gekarakteriseerd door een neoplasma van intra-epitheliale T-cellen dat meestal voorkomt in jejunum of ileum bij patiënten met coeliakie. De laesie kan multifocaal zijn en ulcererende noduli, plaques, stricturen, of een exofytische massa vormen. Mesenterium en mesenterische lymfeklieren zijn meestal betrokken. Patiënten presenteren zich doorgaans met abdominale pijn, malabsorptie of diarree, anorexie, gewichtsverlies, vermoeidheid, misselijkheid, braken, en soms perforatie of hemorragie van darm. De prognose is doorgaans slecht."},"pt":{"name":"Linfoma de células T associado a enteropatia","synonyms":["EATL","ETTL","Linfoma de células T associado a enteropatia tipo 1","Linfoma de células T intestinal","Linfoma de células T, tipo enteropático"]},"pl":{"name":"Chłoniak T-komórkowy enteropatyczny","synonyms":["EATL","Enteropathy-type T-cell lymphoma","ETTL","Jelitowy chłoniak z komórek T","Chłoniak z komórek T związany z enteropatią"]},"cs":{"name":"T-buněčný lymfom asociovaný s enteropatií","synonyms":["EATL","ETTL","T-buněčný lymfom typu enteropatie","Intestinální T-buněčný lymfom"]},"uk":{"name":"Т-клітинна лімфома, асоційована з ентеропатією","synonyms":["EATL","Т-клітинна лімфома, асоційована з ентеропатією, тип 1","Т-клітинна лімфома типу ентеропатії","ETTL","Кишкова Т-клітинна лімфома"],"definition":"A rare T-cell non-Hodgkin lymphoma characterized by a neoplasm of intraepithelial T-cells mostly occurring in the jejunum or ileum in patients with celiac disease. The lesion may be multifocal and form ulcerating nodules, plaques, strictures, or an exophytic mass. The mesentery and mesenteric lymph nodes are commonly involved. Patients typically present with abdominal pain, malabsorption or diarrhea, anorexia, weight loss, fatigue, nausea, vomiting, and sometimes intestinal perforation or hemorrhage. Prognosis is generally poor."},"zh":{"name":"肠病相关T细胞淋巴瘤","synonyms":["肠病型T-细胞淋巴瘤"]},"tr":{"name":"Enteropati-ilişkili T-hücreli lenfoma","synonyms":["EATL","ETTL","Bağırsak T hücreli lenfoma","Enteropati tipi T hücreli lenfoma"]}},"xrefs":[{"source":"ICD-10","code":"C86.2","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"2A90.7","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"958629729"},{"source":"MONDO","code":"0019473","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D058527","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0456889","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"9809","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10073481","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":423793,"name":"Rare tumor of small intestine","classifications":[152,202,233]},{"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":0.0111,"area":"United States","status":"Validated","source":"26104061[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.1,"area":"Netherlands","status":"Validated","source":"18618372[PMID]"}],"phenotypes":null}