{"orpha":542323,"name":"T-cell based immunotherapy-associated cytokine release syndrome","type":"Particular clinical situation in a disease or syndrome","group":"Disorder","status":"active","flags":[],"synonyms":["T-cell based immunotherapy-associated CRS"],"definition":"A rare systemic condition affecting patients undergoing chimeric antigen receptor (CAR) T-cell therapy and characterized by a systemic inflammatory response due to massive activation of leukocytes with subsequent cytokine release. It can present with a variety of signs and symptoms ranging from mild, flu-like symptoms (such as fever, fatigue, headache, rash, arthralgia, and myalgia) to severe life-threatening manifestations including vascular leakage, disseminated intravascular coagulation, shock, and multiple organ failure. Respiratory manifestations are common and range from cough and tachypnea to acute respiratory distress syndrome (ARDS).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-542323/","orphanet_url":"https://www.orpha.net/en/disease/detail/542323","names":{"fr":{"name":"Syndrome de libération de cytokines lié à l'immunothérapie liée aux lymphocytes T","synonyms":["Syndrome de relargage cytokinique lié à l'immunothérapie liée aux lymphocytes T"],"definition":"Maladie systémique rare affectant les patients sous thérapie par lymphocytes T à récepteur antigénique chimérique (CAR-T) et caractérisée par une réponse inflammatoire systémique due à une activation massive des leucocytes avec libération ultérieure de cytokines. Elle peut se manifester par de nombreux signes et symptômes allant de symptômes bénins similaires à ceux d'une grippe (fièvre, fatigue, maux de tête, éruptions cutanées, arthralgie et myalgie) à des manifestations graves mettant en jeu le pronostic vital, notamment une fuite capillaire, une coagulation intravasculaire disséminée, un choc et une défaillance multiviscérale. Les manifestations respiratoires sont fréquentes et vont d'une toux et d'une tachypnée au syndrome de détresse respiratoire aiguë (SDRA)."},"de":{"name":"Zytokin-Freisetzungssyndrom nach T-Zell-basierter Immuntherapie","synonyms":["T-Zell-basierte Immuntherapie-assoziiertes CRS"],"definition":"Eine seltene systemische Erkrankung, die bei Patienten auftritt, die sich einer Therapie mit chimären Antigenrezeptoren (CAR) T-Zellen unterziehen, und die durch eine systemische Entzündungsreaktion aufgrund einer massiven Aktivierung von Leukozyten mit anschließender Freisetzung von Zytokinen gekennzeichnet ist. Sie kann sich mit einer Vielzahl von Anzeichen und Symptomen äußern, die von leichten, grippeähnlichen Symptomen (wie Fieber, Müdigkeit, Kopfschmerzen, Hautausschlag, Arthralgie und Myalgie) bis hin zu schweren, lebensbedrohlichen Manifestationen wie Gefäßleckagen, disseminierter intravaskulärer Gerinnung, Schock und Multiorganversagen reichen. Atemwegsmanifestationen sind häufig und reichen von Husten und Tachypnoe bis zum akuten Atemnotsyndrom (ARDS)."},"es":{"name":"Síndrome de liberación de citocinas asociado a inmunoterapia basada en células T","synonyms":["SLC asociado a inmunoterapia basada en células T"],"definition":"Es una enfermedad sistémica poco frecuente que afecta a los pacientes sometidos a terapia de células T con receptores de antígenos quiméricos (CAR) y que se caracteriza por una respuesta inflamatoria sistémica debida a la activación masiva de los leucocitos con la consiguiente liberación de citoquinas. Puede presentarse con una variedad de signos y síntomas que van desde síntomas de tipo gripal (como fiebre, cansancio, cefalea, erupción cutánea, artralgia y mialgia) hasta manifestaciones graves que ponen en peligro la vida del paciente, como fuga venosa, coagulación intravascular diseminada, shock y fallo multiorgánico. Las manifestaciones respiratorias son comunes y varían desde tos y taquipnea hasta un síndrome de dificultad respiratoria aguda (SDRA)."},"it":{"name":"Sindrome da rilascio di citochine associata all'immunoterapia basata sulle cellule T","synonyms":["CRS associata all'immunoterapia basata sulle cellule T"]},"nl":{"name":"'Cytokine release'-syndroom door immuuntherapie met T-cellen","synonyms":["CRS door immuuntherapie met T-cellen"],"definition":"Een zeldzame systemische aandoening bij patiënten die therapie met T-cellen met chimere antigeenreceptor (CAR) ondergaan, gekenmerkt door systemische inflammatoire respons als gevolg van massale activatie van leukocyten met vervolgens vrijzetting van cytokine. De aandoening kan zich presenteren met een waaier van verschijnselen en symptomen, gaande van milde griepachtige symptomen (zoals koorts, vermoeidheid, hoofdpijn, uitslag, artralgie, en myalgie) tot ernstige levensbedreigende manifestaties waaronder vasculaire lekkage, gedissemineerde intravasculaire coagulatie, shock, en multiorgaanfalen. Respiratoire manifestaties zijn gangbaar, en gaan van hoest en tachypneu tot acute ademnood-syndroom (ARDS)."},"pt":{"name":"Síndrome de libertação de citoquinas associada a terapia com células T CAR","definition":"Doença sistémica rara que afeta doentes submetidos à terapia com células T com recetor de antigenio quimérico (CAR) e caracterizada por uma resposta inflamatória sistémica devido à ativação maciça de leucócitos com subsequente libertação de citocinas. Existe variedade de sinais e sintomas, que variam de leves semelhantes aos da gripe (como febre, fadiga, dor de cabeça, erupção cutânea, artralgia e mialgia) até manifestações graves com risco de vida, incluindo ruptura vascular, coagulação intravascular disseminada, choque, e falência de múltiplos órgãos. As manifestações respiratórias são comuns e variam desde tosse e taquipneia até síndrome de dificuldade respiratória aguda (SDRA)."},"pl":{"name":"Zespół uwalniania cytokin związany z terapią limfocytami T CAR","synonyms":["T-cell based immunotherapy-associated CRS"],"definition":"Rzadki stan ogólnoustrojowy dotykający pacjentów poddawanych terapii limfocytami T z użyciem chimerowego receptora antygenowego (CAR), charakteryzujący się ogólnoustrojową reakcją zapalną z powodu masowej aktywacji leukocytów z następowym uwalnianiem cytokin. Może dawać różne objawy, od łagodnych grypopodobnych (takich jak gorączka, zmęczenie, ból głowy, wysypka, bóle stawów i mięśni) do ciężkich zagrażających życiu, w tym przecieków naczyniowych, rozsianego wykrzepiania wewnątrznaczyniowego, wstrząsu, i niewydolności wielonarządowej. Objawy ze strony układu oddechowego są częste i obejmują spektrum od kaszlu i przyspieszonego oddechu po zespół ostrej niewydolności oddechowej (ARDS)."},"cs":{"name":"Syndrom uvolnění cytokinů spojený s CAR T-buněčnou terapií","synonyms":["T-cell based immunotherapy-associated CRS"]},"uk":{"name":"Синдром вивільнення цитокінів, асоційований з терапією Т-клітинною терапією CAR","synonyms":["CRS, асоційована з CAR Т-клітинною терапією","Синдром вивільнення цитокінів, асоційований з терапією Т-клітинними рецепторами до химерних антигенів"],"definition":"A rare systemic condition affecting patients undergoing chimeric antigen receptor (CAR) T-cell therapy and characterized by a systemic inflammatory response due to massive activation of leukocytes with subsequent cytokine release. It can present with a variety of signs and symptoms ranging from mild, flu-like symptoms (such as fever, fatigue, headache, rash, arthralgia, and myalgia) to severe life-threatening manifestations including vascular leakage, disseminated intravascular coagulation, shock, and multiple organ failure. Respiratory manifestations are common and range from cough and tachypnea to acute respiratory distress syndrome (ARDS)."},"zh":{"name":"CAR T细胞治疗相关的细胞因子释放综合征","synonyms":["嵌合抗原受体-T细胞治疗相关的细胞因子释放综合征"]},"tr":{"name":"CAR T hücre terapisi-ilişkili sitokin salınım sendromu","synonyms":["CAR T hücresi terapisi ile ilişkili CRS","Kimerik antijen reseptörü-T hücre terapisi ile ilişkili sitokin salınım sendromu"]}},"xrefs":[{"source":"ICD-10","code":"D89.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"4A4Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"UMLS","code":"C5680162","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":182222,"name":"Rare systemic disease","classifications":[196]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2021-06-03","source":"30753567[PMID]_30181581[PMID]_28925994[PMID]","terms":[{"hpo":"HP:0011117","label":"Abnormal circulating interleukin concentration","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0011112","label":"Abnormality of serum cytokine level","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012649","label":"Increased inflammatory response","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002615","label":"Hypotension","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030783","label":"Increased circulating interleukin 6 concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030356","label":"Increased serum interferon-gamma level","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003326","label":"Myalgia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0004396","label":"Poor appetite","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001649","label":"Tachycardia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011675","label":"Arrhythmia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030005","label":"Capillary leak","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001289","label":"Confusion","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011037","label":"Decreased urine output","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002014","label":"Diarrhea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003259","label":"Elevated circulating creatinine concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002910","label":"Elevated circulating hepatic transaminase concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012722","label":"Heart block","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002904","label":"Hyperbilirubinemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012418","label":"Hypoxemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002018","label":"Nausea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002202","label":"Pleural effusion","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100598","label":"Pulmonary edema","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012664","label":"Reduced left ventricular ejection fraction","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002789","label":"Tachypnea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002013","label":"Vomiting","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001919","label":"Acute kidney injury","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0005521","label":"Disseminated intravascular coagulation","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002878","label":"Respiratory failure","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000988","label":"Skin rash","frequency":"Very rare (<4-1%)","criteria":null}]}}