{"orpha":70568,"name":"Post-transplant lymphoproliferative disease","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["PTLD"],"definition":"A group of rare immunodeficiency-associated lymphoproliferative disorders characterized by lymphoid or plasmacytic proliferations developing in the context of immunosuppression in a recipient of a solid organ or stem cell allograft. The group includes non-destructive post-transplant lymphoproliferative disorders (PTLDs), polymorphic PTLD, monomorphic PTLDs, and classic Hodgkin lymphoma PTLD. Patients may have more than one type of PTLD in a single or in different locations. The most commonly involved sites are lymph nodes, gastrointestinal tract, lungs, and liver, although the disease may occur almost anywhere in the body. In solid organ transplant recipients, PTLD may also involve the allograft.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-70568/","orphanet_url":"https://www.orpha.net/en/disease/detail/70568","names":{"fr":{"name":"Maladie lymphoproliférative post-tranplantation","definition":"Groupe rare de pathologies immunodéficientes associées à un syndrome lymphoprolifératif caractérisé par des proliférations lymphoïdes ou plasmactiques se développant dans le cadre de l'immunosuppression chez un receveur d'un organe solide ou d'une allogreffe de cellules souches. Ce groupe comprend les maladies lymphoprolifératives post-transplantation (LPT) non destructeurs, les LPT polymorphes, les LPT monomorphes et les LPT classiques liés au lymphome de Hodgkin. Les patients peuvent présenter un ou plusieurs types de LPT touchant une ou plusieurs parties du corps. Les ganglions lymphatiques, le tractus gastro-intestinal, les poumons et le foie sont les parties les plus touchées, mais la maladie peut affecter l'ensemble du corps. Chez les receveurs de greffes d'organes solides, le LPT peut également impliquer l'allogreffe."},"de":{"name":"Lymphoproliferative Erkrankung nach Transplantation","synonyms":["Posttransplantations- Lymphoproliferativen Erkrankung (PTLD)"],"definition":"Eine Gruppe seltener Immundefekt-assoziierter lymphoproliferativer Erkrankungen, die durch lymphoide oder plasmatische Proliferationen gekennzeichnet sind, die sich im Rahmen einer Immunsuppression bei einem Empfänger eines Allotransplantats eines festen Organs oder von Stammzellen entwickeln. Zu dieser Gruppe gehören nicht-destruktive lymphoproliferative Posttransplantationsstörungen (PTLD), polymorphe PTLD, monomorphe PTLD und klassische Hodgkin-Lymphom-PTLD. Die Patienten können mehr als einen Typ von PTLD an einem einzigen oder an verschiedenen Orten haben. Am häufigsten sind die Lymphknoten, der Magen-Darm-Trakt, die Lunge und die Leber betroffen, obwohl die Krankheit fast überall im Körper auftreten kann. Bei Empfängern von Transplantaten fester Organe kann die PTLD auch das Allotransplantat betreffen."},"es":{"name":"Enfermedad linfoproliferativa post-trasplante","definition":"Es un grupo de trastornos linfoproliferativos poco frecuentes asociados a inmunodeficiencia y caracterizado por proliferaciones linfoides o plasmocíticas que se desarrollan en el contexto de inmunosupresión en un receptor de un aloinjerto de órgano sólido o de células madre. El grupo incluye los trastornos linfoproliferativos postrasplante (TLPT) no destructivos, TLPT polimórficos, TLPT monomórficos y TLPT clásico de linfoma de Hodgkin. Los pacientes pueden tener más de un tipo de TLPT en un solo sitio o en diferentes sitios anatómicos. Los sitios más comúnmente involucrados son los ganglios linfáticos, el tracto digestivo, los pulmones y el hígado, aunque la enfermedad puede ocurrir en casi cualquier parte del organismo. En los receptores de trasplantes de órganos sólidos, el TLPT también puede afectar al aloinjerto."},"it":{"name":"Malattia linfoproliferativa post-trapianto","definition":"Si tratta di un gruppo di malattie linfoproliferative rare correlate all'immunodeficienza, caratterizzate dalla proliferazione di linfoidi o plasmacellule che si sviluppano nel quadro dell'immunosoppressione in un paziente che ha ricevuto un allotrapianto di un organo solido o di cellule staminali. Il gruppo comprende le malattie linfoproliferative post-trapianto (PTLD) non distruttive, le PTLD polimorfe, le PTLD monomorfe e le PTLD classiche correlate al linfoma di Hodgkin. I pazienti possono presentare più tipi di PTLD in un'unica sede o in sedi diverse. I siti maggiormente interessati sono i linfonodi, il tratto gastrointestinale, i polmoni e il fegato, ma la malattia può localizzarsi in qualsiasi area del corpo. Nei riceventi il trapianto di organo solido, la PTLD può interessare anche l'allotrapianto."},"nl":{"name":"Post-transplantatie lymfoproliferatieve aandoening","synonyms":["PTLD"],"definition":"Een groep van zeldzame lymfoproliferatieve aandoeningen geassocieerd met immuundeficiëntie, gekarakteriseerd door lymfoïde of plasmacytaire proliferaties die ontwikkelen in de context van immuunsuppressie in een ontvanger van een solide orgaan of allogeen stamceltransplantaat. De groep omvat niet-destructieve lymfoproliferatieve aandoeningen na transplantatie (PTLD's), polymorfe PTLD, monomorfe PTLD's, en klassiek Hodgkinlymfoom PTLD. Patiënten kunnen meer dan een type van PTLD vertonen, op een enkele plaats of op verschillende plaatsen. Plaatsen die het vaakst betrokken zijn, zijn lymfeklieren, maag-darmstelsel, longen, en lever, maar de ziekte kan bijna overal in het lichaam optreden. Bij ontvangers van een solide orgaan kan PTLD ook het allotransplantaat aantasten."},"pt":{"name":"Doença linfoproliferativa pós-transplante","synonyms":["DLPT"]},"pl":{"name":"Potransplantacyjna choroba limfoproliferacyjna","synonyms":["PTLD"]},"cs":{"name":"Potransplantační lymfoproliferativní choroba","synonyms":["PTLCH"]},"uk":{"name":"Посттрансплантаційне лімфопроліферативне захворювання","synonyms":["PTLD"],"definition":"A group of rare immunodeficiency-associated lymphoproliferative disorders characterized by lymphoid or plasmacytic proliferations developing in the context of immunosuppression in a recipient of a solid organ or stem cell allograft. The group includes non-destructive post-transplant lymphoproliferative disorders (PTLDs), polymorphic PTLD, monomorphic PTLDs, and classic Hodgkin lymphoma PTLD. Patients may have more than one type of PTLD in a single or in different locations. The most commonly involved sites are lymph nodes, gastrointestinal tract, lungs, and liver, although the disease may occur almost anywhere in the body. In solid organ transplant recipients, PTLD may also involve the allograft."},"zh":{"name":"移植后淋巴增殖性疾病","synonyms":["PTLD"]},"tr":{"name":"Nakil sonrası lenfoproliferatif hastalık","synonyms":["PTLD"]}},"xrefs":[{"source":"ICD-10","code":"D47.9","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0019088","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0432487","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"9553","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10051358","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":98290,"name":"Immunodeficiency-associated lymphoproliferative disease","classifications":[194,202,233]},{"orpha":171918,"name":"T-cell non-Hodgkin lymphoma","classifications":[194,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-5 / 10 000","value":26.2,"area":"Europe","status":"Validated","source":"European Medicines Agency[OTHER]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2024-03-11","source":"30020621[PMID]_37294957[PMID]_23802715[PMID]","terms":[{"hpo":"HP:0001608","label":"Abnormality of the voice","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002716","label":"Lymphadenopathy","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0033834","label":"Malaise","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030166","label":"Night sweats","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0033050","label":"Pharyngalgia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0004396","label":"Poor appetite","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0025267","label":"Snoring","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002014","label":"Diarrhea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002239","label":"Gastrointestinal hemorrhage","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012115","label":"Hepatitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003073","label":"Hypoalbuminemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0033608","label":"Pulmonary nodule","frequency":"Occasional (29-5%)","criteria":null}]}}