{"orpha":275944,"name":"Hemolytic disease of the newborn with Kell alloimmunization","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Anti-K HDN","Maternal anti-Kell alloimmunization"],"definition":"A rare hematologic disease characterized by the transfer of maternal alloantibodies against red blood cell antigens of the Kell family to a fetus positive for this antigen across the placental barrier, causing suppression of erythropoiesis with reticulocytopenia and anemia, as well as alloimmune hemolysis. Severe anemia may lead to hydrops fetalis. Significant hyperbilirubinemia is rare in this condition.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-275944/","orphanet_url":"https://www.orpha.net/en/disease/detail/275944","names":{"fr":{"name":"Maladie hémolytique du nouveau-né avec allo-immunisation Kell","synonyms":["Allo-immunisation foetomaternelle anti-Kell"],"definition":"Maladie hématologique rare caractérisée par le transfert à travers la barrière placentaire d'allo-anticorps maternels, dirigés contre les antigènes des globules rouges de la famille de Kell à un foetus positif pour cet antigène, provoquant une suppression de l'érythropoïèse avec une réticulocytopénie, une anémie, ainsi qu'une hémolyse allo-immune. Une anémie sévère peut conduire à une anasarque foetale. Une hyperbilirubinémie significative est rare dans cette maladie."},"de":{"name":"Hämolytische Krankheit des Neugeborenen mit Kell-Alloimmunisierung","synonyms":["Anti-Kell HDN","Anti-Kell-Alloimmunisierung, maternale"],"definition":"Eine seltene hämatologische Erkrankung, die durch die Übertragung von mütterlichen Alloantikörpern gegen Erythrozytenantigene der Kell-Familie über die Plazentaschranke auf einen für dieses Antigen positiven Fötus gekennzeichnet ist und eine Hemmung der Erythropoese mit Retikulozytopenie und Anämie sowie eine Alloimmunhämolyse verursacht. Schwere Anämie kann zu Hydrops fetalis führen. Eine signifikante Hyperbilirubinämie ist bei dieser Erkrankung selten."},"es":{"name":"Enfermedad hemolítica neonatal con aloinmunización Kell","synonyms":["Aloinmunización anti-Kell materna","Anti-K HDN"],"definition":"Es una enfermedad hematológica poco frecuente caracterizada por la transferencia de aloanticuerpos maternos contra antígenos del sistema Kell que, a través de la barrera placentaria, actúan contra hematíes fetales positivos para estos, provocando la inhibición de la eritropoyesis con anemia y reticulocitopenia, así como una hemólisis aloinmune sin hiperbilirrubinemia significativa. La forma grave de esta anemia puede resultar en hidropesía fetal."},"it":{"name":"Malattia emolitica del neonato con alloimmunizzazione di Kell","synonyms":["Alloimmunizzazione materna anti-Kell"],"definition":"Si tratta di una malattia ematologica rara, caratterizzata dal passaggio attraverso la placenta degli alloanticorpi materni diretti contro gli antigeni degli eritrociti della famiglia di Kell verso il feto positivo per questo antigene. Ciò causa la soppressione dell'eritropoiesi con reticolocitopenia, anemia ed emolisi alloimmune. L'anemia grave può esitare nell'idrope fetale. Raramente si osserva un'iperbilirubinemia significativa."},"nl":{"name":"Hemolytische ziekte van de neonaat met Kell allo-immunisatie","synonyms":["Anti-K HDN","Maternale anti-Kell allo-immunisatie"],"definition":"Een zeldzame hematologische ziekte, gekarakteriseerd door transfer door de placentabarrière van maternale allo-antilichamen tegen Kell-antigenen van rode bloedcellen naar een foetus die positief is voor dit antigen, wat leidt tot suppressie van erytropoëse met reticulocytopenie en anemie, alsook allo-immune hemolyse. Ernstige anemie kan leiden tot hydrops foetalis. Significante hyperbilirubinemie komt zelden voor bij deze aandoening."},"pt":{"name":"Doença hemolítica do recém-nascido com aloimunização Kell"},"pl":{"name":"Choroba hemolityczna u noworodka z alloimmunizacją Kell","synonyms":["Matczyna alloimmunizacja anty-Kell"]},"cs":{"name":"Hemolytická nemoc novorozence s Kell aloimunizací","synonyms":["Kell aloimunizace matky"]},"uk":{"name":"Гемолітична хвороба новонароджених з алоімунізацією Келла","synonyms":["Anti-K HDN","Материнська алоімунізація проти Келл"],"definition":"A rare hematologic disease characterized by the transfer of maternal alloantibodies against red blood cell antigens of the Kell family to a fetus positive for this antigen across the placental barrier, causing suppression of erythropoiesis with reticulocytopenia and anemia, as well as alloimmune hemolysis. Severe anemia may lead to hydrops fetalis. Significant hyperbilirubinemia is rare in this condition."},"zh":{"name":"Kell同种异体免疫所致新生儿溶血病","synonyms":["母体抗Kell同种异体免疫"],"definition":"一种罕见的血液病，其特征是母体对Kell家族红血球抗原的同种异体抗体通过胎盘屏障转移给该抗原阳性的胎儿，导致伴网织红细胞减少和贫血的红细胞生成抑制，以及同种免疫溶血。严重贫血可能导致胎儿水肿。在这种情况下，明显的高胆红素血症是罕见的。"},"tr":{"name":"Kell alloimmünizasyon ile seyreden yenidoğanın hemolitik hastalığı","synonyms":["Anti-K HDN","Maternal anti-Kell alloimmünizasyon"]}},"xrefs":[{"source":"ICD-10","code":"P55.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"KA84.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0017164","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0472751","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97992,"name":"Rare hematologic disease"},"parents":[{"orpha":275938,"name":"Hemolytic disease due to fetomaternal alloimmunization","classifications":[194]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Infancy","Neonatal"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":">1 / 1000","value":116.0,"area":"United Kingdom","status":"Validated","source":"2081378[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}