{"orpha":86882,"name":"Hepatosplenic T-cell lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare T-cell non-Hodgkin lymphoma characterized by a proliferation of cytotoxic T-cells, usually gamma delta T-cells, with involvement of the liver and spleen, but without involvement of lymph nodes. The bone marrow is consistently affected. Patients typically present during adolescence or young adulthood with hepatosplenomegaly, pancytopenia, and systemic symptoms. Peripheral blood involvement may develop later in the disease course. There is a clear male preponderance. The disease often occurs in the context of long-term immunosuppression. The course is aggressive with poor therapy response.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-86882/","orphanet_url":"https://www.orpha.net/en/disease/detail/86882","names":{"fr":{"name":"Lymphome T hépatosplénique","definition":"Lymphome T non hodgkinien rare caractérisé par une prolifération de cellules T cytotoxiques, généralement des cellules T gamma delta, avec une atteinte du foie et de la rate, mais sans atteinte des ganglions lymphatiques. La moelle osseuse est systématiquement touchée. Les patients manifestent généralement à l'adolescence ou au début de l'âge adulte une hépatosplénomégalie, une pancytopénie et des symptômes systémiques. Plus tard, au cours de l'évolution de la maladie, on peut observer une atteinte du sang périphérique. La maladie est à prépondérance nettement masculine. Elle se manifeste souvent dans un contexte d'immunosuppression à long terme. L'évolution est agressive et la réponse thérapeutique est faible."},"de":{"name":"T-Zell-Lymphom, hepatosplenisches","definition":"Ein seltenes T-Zell-Non-Hodgkin-Lymphom, das durch eine Vermehrung zytotoxischer T-Zellen, in der Regel Gamma-Delta-T-Zellen, mit Befall von Leber und Milz, aber ohne Befall von Lymphknoten gekennzeichnet ist. Das Knochenmark ist durchweg betroffen. Die Patienten stellen sich typischerweise im Jugend- oder jungen Erwachsenenalter mit Hepatosplenomegalie, Panzytopenie und systemischen Symptomen vor. Eine Beteiligung des peripheren Blutes kann sich später im Krankheitsverlauf entwickeln. Männer sind deutlich häufiger betroffen. Die Erkrankung tritt häufig im Zusammenhang mit einer langfristigen Immunsuppression auf. Der Verlauf ist aggressiv mit schlechtem Therapieansprechen."},"es":{"name":"Linfoma hepatoesplénico de células T","definition":"Es un linfoma no Hodgkin de células T poco frecuente caracterizado por la proliferación de células T citotóxicas, por lo general células T gamma delta, con afectación hepática y esplénica, aunque sin afectación de los ganglios linfáticos. La médula ósea se ve afectada habitualmente. La enfermedad debuta típicamente en la adolescencia o en adultos jóvenes con hepatoesplenomegalia, pancitopenia y síntomas sistémicos. La afectación de la sangre periférica puede desarrollarse más tarde en el curso de la enfermedad. Existe un claro predominio masculino. La enfermedad a menudo ocurre en el contexto de una inmunosupresión a largo plazo. El curso es agresivo con una mala respuesta al tratamiento."},"it":{"name":"Linfoma epatosplenico a cellule T","definition":"Si tratta di un linfoma non-Hodgkin a cellule T raro, caratterizzato dalla proliferazione di cellule T citotossiche, di solito cellule T gamma delta, che interessa il fegato e la milza, senza coinvolgere i linfonodi. È molto comune l'interessamento del midollo osseo. Di solito esordisce durante l'adolescenza o la prima età adulta con epatosplenomegalia, pancitopenia e sintomi sistemici. Il sangue periferico può essere interessato successivamente. Si osserva una marcata preponderanza di maschi affetti. Spesso la malattia insorge nel quadro di un'immunosoppressione a lungo termine. Il decorso è aggressivo e la risposta alla terapia è scarsa."},"nl":{"name":"Hepatosplenisch T-cellymfoom","definition":"Een zeldzaam non-Hodgkinlymfoom van T-cellen, gekarakteriseerd door proliferatie van cytotoxische T-cellen, meestal gamma-delta-T-cellen, met betrokkenheid van lever en milt, maar zonder betrokkenheid van lymfeklieren. Beenmerg is altijd aangetast. Patiënten presenteren zich doorgaans tijdens de adolescentie of jonge volwassenheid met hepatosplenomegalie, pancytopenie, en systemische symptomen. Betrokkenheid van perifeer bloed kan zich in een latere fase van het ziekteverloop ontwikkelen. Er is een duidelijk overwicht van mannen. De ziekte komt vaak voor in de context van langdurige immuunsuppressie. De ziekte vertoont een agressief verloop en weinig respons op therapie."},"pt":{"name":"Linfoma hepatoesplénico de células T"},"pl":{"name":"Wątrobowo-śledzionowy chłoniak T-komórkowy"},"cs":{"name":"Hepatosplenický T-lymfom"},"uk":{"name":"Гепатоспленоцитарна Т-клітинна лімфома","definition":"A rare T-cell non-Hodgkin lymphoma characterized by a proliferation of cytotoxic T-cells, usually gamma delta T-cells, with involvement of the liver and spleen, but without involvement of lymph nodes. The bone marrow is consistently affected. Patients typically present during adolescence or young adulthood with hepatosplenomegaly, pancytopenia, and systemic symptoms. Peripheral blood involvement may develop later in the disease course. There is a clear male preponderance. The disease often occurs in the context of long-term immunosuppression. The course is aggressive with poor therapy response."},"zh":{"name":"肝脾T细胞淋巴瘤"},"tr":{"name":"Hepatosplenik T-hücreli lenfoma"}},"xrefs":[{"source":"ICD-10","code":"C86.1","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"2A90.8","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1301206942"},{"source":"MONDO","code":"0019474","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1333984","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10066957","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":["Adolescent","Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.03,"area":"United States","status":"Validated","source":"21823196[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}