Professor Kyung Hee University Hospital at Gangdong Seoul, Seoul-t'ukpyolsi, Republic of Korea
Background: Acquired bleeding disorder is very rare in adolescents and young adults (AYAs) but potentially life-threatening clinical syndrome characterized by the sudden onset of bleeding in patients with a negative family and personal history.
Aims: We report two AYA patients with unusual clinical manifestations, who were diagnosed with acquired bleeding disorders.
Methods: We reviewed two patients who were diagnosed and treated in our clinics retrospectively.
Results: The first case was 19-year-old women with right lower leg painful swelling. She had no past and family history of bleeding disorder. Initial laboratory findings showed prolonged activated partial thromboplastin time (aPTT) and uncorrected mixing test. Factor VIII (FVIII) activity was below 1% and FVIII antibody was 22.4 Bethesda unit. Her diagnosis was acquired hemophilia A. Initial hemostatic treatments started with recombinant activated factor VII and oral steroid as immunosuppression therapy started. After 9 months, FVIII antibody was negative and FVIII activity was normalized. The second case was 17-year-old women with acquired von Willebrand syndrome (AVWS). She also had no past and family history of bleeding disorder. Initially, she was diagnosed with congenital von Willebrand disease. But when she visited the hospital again due to swelling and pain in both hands and wrists, the mixing aPTT was prolonged and low von Willebrand factor antigen (VWF:Ag) and VWF ristocetin cofactor activity (VWF:RCo) were low even after administration of coagulation factors. Her additional laboratory findings were hypoalbuminemia, proteinuria, and low C3 level. She was diagnosed with lupus nephritis and AVWS. She is being monitored with immunosuppressions, but aPTT is still prolonged and low VWF:Ag and VWF:RCo are low.
Conclusion(s): Acquired bleeding disorders are very rare in AYAs, but require a high index of suspicion and close collaboration with laboratories for specialized coagulation testing. An early diagnosis of acquired bleeding disorders is mandatory for starting the appropriate treatment.
