Coexistence of Acquired Hemophilia and Antiphospholipid Serology in Monoclonal Gammopathy Patient

Abstract

Acquired hemophilia is a rare coagulopathy with hemorrhage into the skin, muscle, or soft tissues and mucous membranes and caused by inhibitor antibodies, mainly against FVIII. We report a case of acquired hemophilia presenting with diffuse cutaneous hemorrhage and hemothorax. The patient was found to have acquired an FVIII inhibitor and a high titer of anti β2 glycoprotein 1 IgG and IgM, and anticardiolipin IgM in the context of IgA kappa-type monoclonal gammopathy. He received 3 injections of recombinant factor VII (rFVIIa) and blood transfusion. He was started on steroids and oral cyclophosphamide for 6 weeks. Thromboprophylaxis with aspirin at 100 mg/day was started 3 months after discharge. Antiphospholipid antibodies remained positive after 3 months as well as prolonged aPTT, factor VIII raised at 100%, and the inhibitor was not detected. The association between acquired hemophilia and antiphospholipid antibodies is rare and its distinction is mandatory because clinical presentation ranges from massive hemorrhage to thrombosis.

Keywords:

• acquired hemophilia
• factor VIII deficiency
• antiphospholipid antibodies
• hemorrhage
• treatment

Ethics and Consent Statements

A written informed consent has been signed by the patient to have the case details with published images. No institutional approval was required to publish the case details.

Disclosure

The authors report no conflicts of interest in this work.