title: Macrohematuria as initial presentation in a girl with factor VIII and factor IX inhibitors associated with systemic lupus erythematosus.
abstract: Acquired hemophilia is rarely observed in a pediatric population. We report a case of a 14-year-old girl presented with ecchymoses and macrohematuria. She developed factor VIII and factor IX inhibitors, and was diagnosed with simultaneous acquired hemophilia and systemic lupus erythematosus (SLE). Recombinant-activated FVII and corticosteroid were prescribed due to macrohematuria-related hypovolemia and anemia, which resolved satisfactorily. This case is a reminder that the rare concurrent presence of factor VIII and factor IX inhibitors could be associated with SLE in a pediatric population. Children with SLE-associated-acquired hemophilia may develop macrohematuria as well.
authors.affiliation: Division of Pediatric Immunology, Taichung Veterans General Hospital, Taipei, Taiwan.
authors.forename: Chian-Yin
authors.initials: CY
authors.lastname: Shen
authors.forename: Lin-Shien
authors.initials: LS
authors.lastname: Fu
authors.forename: Heng-Kuei
authors.initials: HK
authors.lastname: Lin
authors.forename: Shao-Min
authors.initials: SM
authors.lastname: Han
authors.forename: Jiaan-Der
authors.initials: JD
authors.lastname: Wang
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D001323:Autoantibodies / Q000276:immunology*; D004438:Ecchymosis / Q000209:etiology; D005164:Factor IX / Q000276:immunology*; D005169:Factor VIII / Q000276:immunology*; D015942:Factor VIIa / Q000627:therapeutic use*; D005260:Female; D006417:Hematuria / Q000188:drug therapy / Q000209:etiology*; D006467:Hemophilia A / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D002836:Hemophilia B / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D006801:Humans; D020896:Hypovolemia / Q000209:etiology; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000276:immunology; D010490:Pericardial Effusion / Q000209:etiology; D011994:Recombinant Proteins / Q000627:therapeutic use; D013610:Tachycardia / Q000209:etiology
chemical_list: D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D011994:Recombinant Proteins; D005169:Factor VIII; D005164:Factor IX; C103587:recombinant FVIIa; D015942:Factor VIIa