Spontaneously acquired factor IX inhibitor in a nonhemophiliac child.
A 2 1/2-year-old, previously healthy child developed progressive swelling of the left leg and a hematoma of the anterior chest wall associated with a falling hemoglobin concentration, as a result of a spontaneously acquired Factor IX inhibitor. Successful management of her condition required a four-volume exchange transfusion and immunosuppressive therapy consisting of cyclophosphamide for four days and prednisone for one month. A brief review of the literature regarding the occurrence and nature of acquired coagulation factor inhibitors and the role of immunosuppressive therapy is also presented.
D001777:Blood Coagulation; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000627:therapeutic use; D004438:Ecchymosis / Q000209:etiology / Q000628:therapy; D005078:Exchange Transfusion, Whole Blood; D005164:Factor IX / Q000037:antagonists & inhibitors*; D005260:Female; D002836:Hemophilia B / Q000097:blood / Q000150:complications / Q000209:etiology*; D006801:Humans; D011241:Prednisone / Q000627:therapeutic use
D003520:Cyclophosphamide; D005164:Factor IX; D011241:Prednisone