A Case of Acquired Hemophilia A and Congenital Hemophilia B

Abstract

Congenital hemophilia B is a rare, inherited X-linked bleeding disorder caused by a deficiency of factor IX (FIX). Acquired hemophilia A is a rare, acquired bleeding disorder which presents as new onset bleeding in older adults due to the development of autoantibodies against factor VIII (FVIII). This report describes the management of a patient with congenital hemophilia B and acquired hemophilia A. We highlight the limitations in maintaining FVIII levels using factor replacement alone and the need for escalating treatment such as rituximab and prednisone in patients with acquired hemophilia A. This case demonstrates the importance of continuing to pursue alternative diagnoses when existing ones do not explain the full clinical picture and laboratory data is inconclusive.

Hemophilia B: A Pain in the Back

“…Another reported a case of arm bleeding, which then expanded to the posterior chest wall in a myelodysplastic syndrome patient with acquired hemophilia A [ 10 ]. The only case we found of a chest wall hematoma in a congenital hemophilia B patient occurred in the traumatic setting of a fall, with this patient also having coexisting acquired hemophilia A as well [ 11 ]. To our knowledge, this is the first reported case of a spontaneous lateral chest wall hematoma in a patient with confirmed inherited hemophilia B.…”

“…In the case of new patients, approximately 50-60 international units/kg of factor IX can be given to raise the factor IX level by 50%. Some cases have reported treatment with the addition of immunosuppressive agents such as cyclophosphamide and prednisone [ 9 , 10 ], as well as antibody therapy with emicizumab [ 8 ] and rituximab [ 11 ]. Patients should be monitored for any drops in hemoglobin as well as signs of neurovascular bundle impingement.…”