{"orpha":93585,"name":"Immune-mediated thrombotic thrombocytopenic purpura","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["Acquired TTP","Acquired thrombotic thrombocytopenic purpura","Autoimmune thrombotic thrombocytopenic purpura","Thrombotic thrombocytopenic purpura due to anti-ADAMTS-13 antibodies","aTTP","iTTP"],"definition":"A rare, non-hereditary thrombotic thrombocytopenic purpura (TTP), characterized by profound peripheral thrombocytopenia, microangiopathic hemolytic anemia (MAHA) and single or multiple organ failure of variable severity.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-93585/","orphanet_url":"https://www.orpha.net/en/disease/detail/93585","names":{"fr":{"name":"Purpura thrombotique thrombocytopénique d'origine immunitaire","synonyms":["PTT acquis","Purpura thrombotique thrombocytopénique acquis","Purpura thrombotique thrombocytopénique associé aux anticorps anti-ADAMTS-13","Purpura thrombotique thrombocytopénique d'origine auto-immune","TTP acquis"],"definition":"Purpura thrombotique thrombocytopénique (PTT) non héréditaire rare, caractérisé par une thrombopénie périphérique profonde, une anémie hémolytique microangiopathique (AHMA) et une défaillance de sévérité variable d'un ou de plusieurs organes."},"de":{"name":"Thrombotisch-thrombozytopenische Purpura, immun-vermittelte","synonyms":["Autoimmune thrombotisch-thrombozytopenische Purpura","Erworbene TTP","aTTP","iTTP"],"definition":"Eine nicht hereditäre thrombotische thrombozytopenische Purpura (TTP), die durch eine ausgeprägte periphere Thrombozytopenie, eine mikroangiopathische hämolytische Anämie (MAHA) und ein oder mehrere Organversagen unterschiedlichen Schweregrades gekennzeichnet ist."},"es":{"name":"Púrpura trombótica trombocitopénica inmunomediada","synonyms":["PTT adquirida","Púrpura trombocitopénica trombótica autoinmune","Púrpura trombocitopénica trombótica por anticuerpos anti-ADAMTS-13"],"definition":"Es una forma no hereditaria de la púrpura trombótica trombocitopénica (PTT) caracterizada por intensa trombocitopenia, anemia hemolítica microangiopática (AHMA) e insuficiencia orgánica o multiorgánica de gravedad variable."},"it":{"name":"Porpora trombotica trombocitopenica immuno-mediata","synonyms":["Porpora trombotica trombocitopenica acquisita da anticorpi anti-ADAMTS 13","Porpora trombotica trombocitopenica autoimmune","TTP acquisita"],"definition":"Si tratta di una porpora trombotica trombocitopenica (TTP) rara non ereditaria, caratterizzata da trombocitopenia periferica grave, anemia emolitica microangiopatica (MAHA) ed insufficienza di uno o più organi di gravità variabile."},"nl":{"name":"Immuungemedieerde trombotische trombocytopenische purpura","synonyms":["Auto-immune trombotische trombocytopenische purpura","Trombotische trombocytopenische purpura door anti-ADAMTS-13-antilichamen","Verworven TTP","Verworven trombotische trombocytopenische purpura","aTTP","iTTP"],"definition":"Een zeldzame, niet-hereditaire trombotische trombocytopenische purpura (TTP), gekarakteriseerd door ernstige perifere trombocytopenie, microangiopathische hemolytische anemie (MAHA), en enkel- of meervoudig orgaanfalen met variabele ernst."},"pt":{"name":"Púrpura trombocitopénica trombótica imunomediada","synonyms":["Púrpura trombocitopénica trombótica adquirida por anticorpos anti-ADAMTS 13"]},"pl":{"name":"Nabyta plamica zakrzepowa małopłytkowa","synonyms":["Autoimmunologiczna plamica zakrzepowa małopłytkowa","Nabyta TTP"]},"cs":{"name":"Získaná trombotická trombocytopenická purpura","synonyms":["Získaná TTP","Autoimunitní trombotická trombocytopenická purpura"]},"uk":{"name":"Імуноопосередкована тромботична тромбоцитопенічна пурпура","synonyms":["Набута тромботична тромбоцитопенічна пурпура","Набутий ТТП","aTTП","Аутоімунна тромботична тромбоцитопенічна пурпура","iTTП","Тромботична тромбоцитопенічна пурпура, спричинена анти-АДАМТС-13 антитілами"],"definition":"A rare, non-hereditary thrombotic thrombocytopenic purpura (TTP), characterized by profound peripheral thrombocytopenia, microangiopathic hemolytic anemia (MAHA) and single or multiple organ failure of variable severity."},"zh":{"name":"获得性血栓性血小板减少性紫癜","synonyms":["抗ADAMTS抗体所致血栓性血小板减少性紫癜"],"definition":"一种罕见的非遗传性血小板减少性紫癜（TTP），其特征是严重的外周血小板减少、微血管病性溶血性贫血（MAHA）和严重程度不同的单器官或多器官衰竭。"},"tr":{"name":"Edinsel trombotik trombositopenik purpura","synonyms":["Edinsel TTP","Otoimmün trombotik trombositopenik purpura","Anti-ADAMTS-13 antikorlarına bağlı trombotik trombositopenik purpura"]}},"xrefs":[{"source":"ICD-10","code":"M31.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"3B64.14","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"54955327"},{"source":"MONDO","code":"0019740","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"C536901","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2584778","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"4607","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97992,"name":"Rare hematologic disease"},"parents":[{"orpha":54057,"name":"Thrombotic thrombocytopenic purpura","classifications":[188,194,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Multigenic/multifactorial"],"onset":["All ages"],"prevalence":[{"type":"Annual incidence","qualification":"Class only","class":"1-9 / 1 000 000","value":0.0,"area":"Europe","status":"Validated","source":"ORPHANET"},{"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":"2024-02-28","source":"33540569[PMID]_30531363[PMID]_28416507[PMID]_28382967[PMID]_31107120[PMID]","terms":[{"hpo":"HP:6000462","label":"Anti-ADAMTS13 antibody positivity","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001937","label":"Microangiopathic hemolytic anemia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000979","label":"Purpura","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001873","label":"Thrombocytopenia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002829","label":"Arthralgia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001259","label":"Coma","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001289","label":"Confusion","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002014","label":"Diarrhea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002094","label":"Dyspnea","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:0002315","label":"Headache","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000790","label":"Hematuria","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002904","label":"Hyperbilirubinemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0025435","label":"Increased circulating lactate dehydrogenase concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0410173","label":"Increased circulating troponin I concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0033404","label":"Intestinal ischemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003419","label":"Low back pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001324","label":"Muscle weakness","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003326","label":"Myalgia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002018","label":"Nausea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000093","label":"Proteinuria","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0020181","label":"Reduced haptoglobin level","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001923","label":"Reticulocytosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001981","label":"Schistocytosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001250","label":"Seizure","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001297","label":"Stroke","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0008282","label":"Unconjugated hyperbilirubinemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003115","label":"Abnormal EKG","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001919","label":"Acute kidney injury","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002381","label":"Aphasia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011675","label":"Arrhythmia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030149","label":"Cardiogenic shock","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100749","label":"Chest pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001635","label":"Congestive heart failure","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000651","label":"Diplopia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003259","label":"Elevated circulating creatinine concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003138","label":"Increased blood urea nitrogen","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001658","label":"Myocardial infarction","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001645","label":"Sudden cardiac death","frequency":"Occasional (29-5%)","criteria":null}]}}