Leonor Farinha (Portugal)
ARSLVT USF S. Julião de OeirasAuthor Of 1 Presentation
ACQUIRED HEMOPHILIA A - A CURIOUS CASE OF BLEEDING DISORDER
Abstract
Abstract Body
Background and purpose
Acquired hemophilia A (AHA) is a rare bleeding disorder caused by autoantibodies against coagulation factor VIII (FVIII). The hallmark of this condition is spontaneous mucocutaneous or soft tissue hemorrhage in patients with no previous history of bleeding. AHA occurs more frequently in the elderly and about half of the cases are idiopathic.
Methods
In the beginning of 2020, an 82 year old male with multiple comorbidities and polymedicated, developed an atypical and exuberant stasis dermatitis of the lower legs with superficial hemorrhagic lesions difficult to heal even after therapeutic adjustment. Posteriorly, he presented spontaneous gingival bleeding and subsequent episode of macroscopic hematuria, which led to multiple visits to his family physician (FP) and to the emergency room.
Results
Lab evaluation showed a progressive and isolated prolonged activated partial thromboplastin time (aPTT) and an extremely low FVIII activity (<0.1%), suggesting the presence of an inhibitor. In this context, the patient was admitted in order to investigate a possible underlying condition, and the diagnosis of AHA was confirmed. Therapy with prothrombin complex concentrate and oral corticosteroids was initiated, with progressive decrease in aPTT and clinical resolution.
Conclusions
AHA is a complex and heterogeneous disease. Early recognition and prompt initiation of treatment can be life-saving. As the first contact with health care system, the FP has a crucial role in recognizing clinical manifestations and framing them into a possible diagnosis, to an appropriate approach. This report highlights the need of different specialties’ knowledge and contribution for a better management of daily practice challenges.
Presenter of 1 Presentation
ACQUIRED HEMOPHILIA A - A CURIOUS CASE OF BLEEDING DISORDER
Abstract
Abstract Body
Background and purpose
Acquired hemophilia A (AHA) is a rare bleeding disorder caused by autoantibodies against coagulation factor VIII (FVIII). The hallmark of this condition is spontaneous mucocutaneous or soft tissue hemorrhage in patients with no previous history of bleeding. AHA occurs more frequently in the elderly and about half of the cases are idiopathic.
Methods
In the beginning of 2020, an 82 year old male with multiple comorbidities and polymedicated, developed an atypical and exuberant stasis dermatitis of the lower legs with superficial hemorrhagic lesions difficult to heal even after therapeutic adjustment. Posteriorly, he presented spontaneous gingival bleeding and subsequent episode of macroscopic hematuria, which led to multiple visits to his family physician (FP) and to the emergency room.
Results
Lab evaluation showed a progressive and isolated prolonged activated partial thromboplastin time (aPTT) and an extremely low FVIII activity (<0.1%), suggesting the presence of an inhibitor. In this context, the patient was admitted in order to investigate a possible underlying condition, and the diagnosis of AHA was confirmed. Therapy with prothrombin complex concentrate and oral corticosteroids was initiated, with progressive decrease in aPTT and clinical resolution.
Conclusions
AHA is a complex and heterogeneous disease. Early recognition and prompt initiation of treatment can be life-saving. As the first contact with health care system, the FP has a crucial role in recognizing clinical manifestations and framing them into a possible diagnosis, to an appropriate approach. This report highlights the need of different specialties’ knowledge and contribution for a better management of daily practice challenges.