A case of acquired hemophilia A in an elderly female

Abstract

Acquired Hemophilia A (AHA) is a bleeding diathesis related to the development of factor VIII inhibitor, which can frequently go undetected. It commonly manifests as spontaneous mucosal bleeds without any known history of a bleeding disorder, but has the potential to cause life threatening bleeding especially in elderly patients with underlying comorbidities. Here we describe a case of AHA in a 78 year old female presenting with spontaneous mucocutaneous bleeding as tongue hematoma and recurrent gastrointestinal (GI) bleeding. Underlying etiology remained unclear in this case. While she did not require any reversal agents to control bleeding, the patient received steroids and rituximab as inhibitor eradication therapy.

Acquired Hemophilia A: A Rare but Potentially Fatal Bleeding Disorder

“…Unlike individuals of congenital hemophilia, bleeding into the joints is rare [2]. The bleeding can occur spontaneously or can be precipitated by events such as surgery [3,4]. The bleeding presentation of acquired hemophilia can be more severe than the bleeding seen with congenital hemophilia cases because there is a delay in diagnosing and treating the condition [1,2].…”

“…Acquired hemophilia is a disease found predominantly in the elderly population without any prior history of bleeding, such as in our case of the 82-year-old male. Additionally, no difference was seen in the distribution of the disease with regards to sex [1-3]. Researchers think that 50% of the cases are idiopathic.…”

“…Autoimmune causes are due to the formation of factor VIII inhibiting antibodies that inhibit the clotting function of factor VIII leading to the mucosal bleeding. The bleeding associated with AHA tends to be in mucocutaneous sites or soft tissues and can lead to recurrent gastrointestinal, intramuscular, or intracranial bleeding in elderly patients as compared to hemarthrosis in younger patients with congenital hemophilia [2,3]. Diagnosis is made with laboratory tests including coagulation and mixing studies.…”

Acquired Hemophilia A: A Potentially Fatal Bleeding Disorder

“…Most of the cases are idiopathic; however, several risk factors are implicated in AHA, the most common being pregnancy, post-partum status, and autoimmune disorders. The other risk factors include malignancies, dermatological disorders, and medications [7,10]. In our case, the patient did not have any underlying medical conditions and was not on any medication which could be related to AHA.…”

“…In patients with low factor VIII inhibitor titers (<5 BU) replacement with human factor VIII concentrates± DDAVP administration is recommended [3][4][5][6][7][8][9][10][11][12][13]. If the patient's antibody titer is high (>5 BU), replacement with even high doses of human factor VIII concentrates, is ineffective, as the inhibitor will immediately block all the factor VIII administered, therefore these patients are managed with bypassing agents [9].…”