Oral manifestations of acquired hemophilia A. A clinical case report; Manifestaciones orales de la hemofilia adquirida tipo A. Reporte de un caso clínico
In: Revista de sanidad militar: organo oficial de la Dirección General de Sanidad Militar, Band 72, Heft 5-6, S. 355-358
Introduction: Acquired hemophilia
A is a hemorrhagic disorder produced by autoantibodies
directed against circulating factor VIII. Bleeding often
appears in the skin, muscles, the retroperitoneal area, and
the gastrointestinal tract; however, it is not frequent
regarding occurrence in the oral cavity. Case report: We
report an unusual case of an elderly woman in the eighth
decade of life with AHA characterized by painful blood
blisters in the oral cavity. The diagnosis was established
with a high activated partial thromboplastin time (aPTT),
normal prothrombin time and elevated levels of factor VIII
inhibitor (FVIII: C). The patient was treated with the
activated recombinant factor VII and prednisone associated
with cyclophosphamide during six weeks. Drugs were stopped
when normalization of aPTT was identified. Discussion: The
diagnosis of HAA is complicated because there is no family
history of bleeding and its autoimmune etiopathogenesis.
Furthermore, the clinical presentation is due to
spontaneous and severe bleeding associated with a prolonged
time of thromboplastin. Conclusion: The initial
identification and the application of the treatment are
essential to reduce the mortality rate of those patients
affected.