{"orpha":99147,"name":"Acquired von Willebrand syndrome","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Acquired von Willebrand disease"],"definition":"A rare bleeding disorder characterized by defects in von Willebrand factor, similar to those seen in hereditary von Willebrand disease (VWD), but which are caused by an underlying pathology, generally in elderly patients without any personal or family history of bleeding anomalies.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-99147/","orphanet_url":"https://www.orpha.net/en/disease/detail/99147","names":{"fr":{"name":"Syndrome de von Willebrand acquis","synonyms":["Maladie de von Willebrand acquise"],"definition":"Maladie hémorragique rare caractérisée par des anomalies du facteur von Willebrand, similaires à celles de la maladie de von Willebrand, mais dues à une pathologie sous-jacente. La maladie touche généralement les patients âgés sans antécédents hémorragiques personnels ou familiaux."},"de":{"name":"Von-Willebrand-Syndrom, erworbenes","synonyms":["AWVS"],"definition":"Eine seltene Blutgerinnungsstörung, die durch Defekte des von-Willebrand-Faktors gekennzeichnet ist und der hereditären Von-Willebrand-Erkrankung (VWE) ähnelt. Sie wird jedoch durch eine zugrunde liegende Pathologie verursacht und tritt in der Regel bei älteren Patienten ohne persönliche oder familiäre Vorgeschichte von Blutungsanomalien auf."},"es":{"name":"Síndrome de Von Willebrand adquirido","definition":"Es un trastorno hemorrágico poco frecuente caracterizado por defectos del factor de von Willebrand, similares a los observados en la enfermedad de von Willebrand (EVW) hereditaria, pero que están causados por una patología subyacente, generalmente en pacientes de edad avanzada sin antecedentes personales o familiares de hemorragias."},"it":{"name":"Malattia di von Willebrand acquisita"},"nl":{"name":"Verworven syndroom van von Willebrand","synonyms":["Verworven ziekte van Von Willebrand"],"definition":"Een zeldzame stollingsstoornis, gekenmerkt door defecten in von-Willebrand-factor, gelijkaardig aan diegene die worden waargenomen bij erfelijke ziekte van von Willebrand (VWD), maar die worden veroorzaakt door een onderliggende pathologie, doorgaans bij oudere patiënten zonder enige persoonlijke of familiale geschiedenis van stollingsstoornissen."},"pt":{"name":"Doença de von Willebrand adquirida"},"pl":{"name":"Nabyty zespół von Willebranda","synonyms":["Nabyta choroba von Willebranda"]},"cs":{"name":"Získaný von Willebrandův syndrom","synonyms":["Získaná von Willebrandova choroba"]},"uk":{"name":"Набутий синдром фон Віллебранда","synonyms":["Набута хвороба фон Віллебранда"],"definition":"A rare bleeding disorder marked by the same biological anomalies as those seen in hereditary von Willebrand disease (VWD) but which occurs in association with another underlying pathology, generally in elderly patients without any personal or family history of bleeding anomalies."},"zh":{"name":"获得性假血友病综合征","synonyms":["获得性血管性血友病"],"definition":"该病是一种罕见的出血性疾病，与遗传性血管性血友病(VWD)有相同的生理异常，但与另一种潜在病理相关，通常发生在没有任何个人或家族出血病史的老年患者中。"},"tr":{"name":"Edinsel von Willebrand sendromu","synonyms":["Edinsel Von Willebrand hastalığı"]}},"xrefs":[{"source":"ICD-10","code":"D68.4","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"3B2Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"62860395"},{"source":"MONDO","code":"0020460","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0272362","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"5573","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10069495","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97992,"name":"Rare hematologic disease"},"parents":[{"orpha":166775,"name":"Rare hemorrhagic disorder due to an acquired coagulation factor defect","classifications":[194]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":300.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2019-10-08","source":"29239101[PMID]_30057374[PMID]_30214173[PMID]_31004311[PMID]_31293998[PMID]","terms":[{"hpo":"HP:0030680","label":"Abnormal cardiovascular system morphology","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0004377","label":"Hematological neoplasm","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030129","label":"Impaired ristocetin cofactor assay activity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0008151","label":"Prolonged prothrombin time","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003125","label":"Reduced factor VIII activity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0008330","label":"Reduced von Willebrand factor activity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001659","label":"Aortic regurgitation","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001650","label":"Aortic valve stenosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025406","label":"Asthenia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000978","label":"Bruising susceptibility","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000421","label":"Epistaxis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000471","label":"Gastrointestinal angiodysplasia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002239","label":"Gastrointestinal hemorrhage","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000790","label":"Hematuria","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001931","label":"Hypochromic anemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002615","label":"Hypotension","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0005261","label":"Joint hemorrhage","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002249","label":"Melena","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000132","label":"Menorrhagia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100608","label":"Metrorrhagia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001653","label":"Mitral regurgitation","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001897","label":"Normocytic anemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001934","label":"Persistent bleeding after trauma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001642","label":"Pulmonic stenosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0005505","label":"Refractory anemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001933","label":"Subcutaneous hemorrhage","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002170","label":"Intracranial hemorrhage","frequency":"Very rare (<4-1%)","criteria":null}]}}