{"orpha":364033,"name":"Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Systemic EBV+ T-cell LPD of childhood","Systemic EBV-positive T-cell lymphoproliferative disease of childhood"],"definition":"A rare and very aggressive neoplastic disease emerging after a primary acute or chronic active EBV infection. It presents with persisting fever and malaise, hepatosplenomegaly with or without lymphadenopathy, liver failure, severe pancytopenia and a rapid progression towards multi-organ failure and hemophagocytic syndrome with a fatal issue. It is characterized by clonal proliferation of EBV-infected T cells with an activated cytotoxic phenotype.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-364033/","orphanet_url":"https://www.orpha.net/en/disease/detail/364033","names":{"fr":{"name":"Maladie lymphoproliférative systémique à cellules T positive au virus Epstein-Barr chez l'enfant","synonyms":["Maladie lymphoproliférative systémique à cellules T positive à l'EBV chez l'enfant"],"definition":"La maladie lymphoproliférative systémique à cellules T positive au virus Epstein-Barr (EBV) chez l'enfant est une néoplasie rare très agressive, survenant après une infection primaire aigüe ou une infection chronique active par l'EBV. Elle se manifeste par une fièvre persistante et un malaise, une hépatosplénomégalie avec ou sans lymphadénopathie, une insuffisance hépatique, une pancytopénie sévère et une progression rapide vers l'insuffisance de plusieurs organes et un syndrome hémophagocytaire, avec une issue fatale. Elle est caractérisée par la prolifération clonale de lymphocytes T infectés par l'EBV, associée à un phénotype cytotoxique activé."},"de":{"name":"Systemische EBV-positive T-Zell-lymphoproliferative Erkrankung der Kindheit","synonyms":["Systemische EBV+ T-Zell LPD der Kindheit"]},"es":{"name":"Enfermedad linfoproliferativa sistémica de células T asociada al virus Epstein-Barr de la infancia","synonyms":["ELP sistémica de células T EBV-positiva de la infancia","Enfermedad linfoproliferativa sistémica de células T EBV-positivas de la infancia"],"definition":"Es una enfermedad neoplásica poco frecuente y muy agresiva que se desarrolla tras una infección primaria activa aguda o crónica por el VEB. Se presenta con fiebre persistente y malestar, hepatoesplenomegalia con o sin linfadenopatía, insuficiencia hepática, pancitopenia grave y una rápida progresión hacia un fallo multiorgánico y síndrome hemofagocítico con consecuencias fatales. Se caracteriza por la proliferación clonal de células T citotóxicas activadas infectadas por el VEB."},"it":{"name":"Malattia linfoproliferativa delle cellule T Epstein-Barr virus-positiva sistemica dell'infanzia","synonyms":["LPD delle cellule T EBV+ sistemica dell'infanzia","Malattia linfoproliferativa delle cellule T EBV-positiva sistemica dell'infanzia"],"definition":"La malattia linfoproliferativa sistemica delle cellule T dell'infanzia, positiva al virus di Epstein-Barr (EBV), è una neoplasia rara e molto aggressiva che esordisce dopo un'infezione da EBV primaria acuta o cronica. La malattia si manifesta con febbre e malessere persistenti, epatosplenomegalia con o senza linfoadenopatia, insufficienza epatica, pancitopenia grave e rapida evoluzione verso l'insufficienza multiorgano e la sindrome emofagocitica con esito letale. La malattia è caratterizzata dalla proliferazione clonale di cellule T infettate da EBV con un fenotipo citotossico attivato."},"nl":{"name":"Systemische Epstein-Barrvirus-positieve T-cel lymfoproliferatieve ziekte van de kindertijd","synonyms":["Systemische EBV+ T-cel LPD van de kindertijd","Systemische EBV-positieve T-cel lymfoproliferatieve ziekte van de kindertijd"],"definition":"Een zeldzame en zeer agressieve neoplastische aandoening die opkomt na een primaire acute of chronische actieve EBV-infectie. De ziekte presenteert zich met aanhoudende koorts en malaise, hepatosplenomegalie met of zonder lymfadenopathie, leverfalen, ernstige pancytopenie en een snelle progressie naar multi-orgaanfalen en hemofagocytair syndroom met een fatale afloop. De aandoening wordt gekarakteriseerd door klonale proliferatie van EBV-geïnfecteerde T-cellen met een geactiveerd cytotoxisch fenotype."},"pt":{"name":"Doença linfoproliferativa da infância com células T positivas para vírus Epstein-Barr sistémico"},"pl":{"name":"Układowa choroba limfoproliferacyjna z komórek T wirus Ebsteia i Barra pozytywna wieku dzieciecęgo","synonyms":["Układowa choroba limfoproliferacyjna z komórek T EBV pozytywna wieku dzieciecęgo","Układowa LPD z komórek T EBV+ wieku dziecięcego"]},"cs":{"name":"Systémová T-buněčná lymfoproliferativní nemoc dětského věku s pozitivitou viru Epsteina-Barrové","synonyms":["Systémová EBV+ T-buněčná LPD v dětství"]},"uk":{"name":"Системна Епштейн-Барр-вірус-позитивна Т-клітинна лімфопроліферативна хвороба дитячого віку","synonyms":["Системна ЕБВ+ Т-клітинна ЛПЗ дитячого віку","Системна ЕБВ-позитивна Т-клітинна лімфопроліферативна хвороба дитячого віку"],"definition":"A rare and very aggressive neoplastic disease emerging after a primary acute or chronic active EBV infection. It presents with persisting fever and malaise, hepatosplenomegaly with or without lymphadenopathy, liver failure, severe pancytopenia and a rapid progression towards multi-organ failure and hemophagocytic syndrome with a fatal issue. It is characterized by clonal proliferation of EBV-infected T cells with an activated cytotoxic phenotype."},"zh":{"name":"儿童系统性EB病毒阳性-T淋巴细胞增生性疾病","synonyms":["儿童系统性EBV阳性T细胞淋巴增生性疾病"],"definition":"本病是原发性急性或慢性活动性EBV感染后出现的一种罕见且侵袭性极强的肿瘤性疾病。本病表现为持续发热和不适，肝脾肿大伴或不伴淋巴结肿大，肝功能衰竭，严重的全血细胞减少，并迅速进展为多器官衰竭和噬血细胞综合征并伴有致命问题。本病的特征是EBV感染的T细胞克隆性增殖，并具有激活的细胞毒表型。"},"tr":{"name":"Çocukluk çağı sistemik Epstein-Barr virüsü pozitif T hücreli lenfoproliferatif hastalık","synonyms":["Çocukluk çağı sistemik EBV + T-hücreli LPD'si","Çocukluk çağı sistemik EBV pozitif T-hücreli lenfoproliferatif hastalığı"]}},"xrefs":[{"source":"ICD-10","code":"D82.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018223","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2699747","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":289644,"name":"Epstein-Barr virus-associated malignant lymphoproliferative disorder","classifications":[202]},{"orpha":171918,"name":"T-cell non-Hodgkin lymphoma","classifications":[194,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Childhood"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Europe","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}