A Case of Acquired Hemophilia A Diagnosed with Submucosal Hematoma of Oral Cavity

Abstract Background Acquired hemophilia A is a rare coagulopathy caused by inhibitors of blood coagulation factor VIII. Patients with acquired hemophilia A have a higher mortality risk (5–10%) than those with congenital hemophilia. Moreover, there is no established evidence of management recommended for patients with acquired hemophilia A. Previous studies have reported the presence of hematomas in the oral cavities of patients with acquired hemophilia A, which were treated conservatively. Here, we describe the case of a patient with acquired hemophilia A, where emergency surgical hemostasis was required for large intraoral hematomas. Case presentation A 65-year-old Japanese man was referred to our hospital with a chief complaint of bleeding from large intraoral hematomas. On examination, he could not close his mouth because of the hematomas, which were bleeding spontaneously. Computed tomography angiography revealed no evidence of arteriovenous malformation, and blood test results showed that the activated partial thromboplastin time was elevated beyond the normal limit. To avoid a life-threatening hemorrhage from hematomas, emergency surgical hemostasis was performed with nasotracheal intubation using fiberoptic bronchoscopy. Hemostasis was successfully performed, as the hematomas were carefully removed. Moreover, the clinical course was successfully completed using intravenously administered activated prothrombin complex concentrate for hemostasis after operation. Conclusions Acquired hemophilia A can cause a life-threatening hemorrhage without predictive factors. Intraoral hematoma may cause airway obstruction. There is no consensus regarding the management of hemorrhage in patients with acquired hemophilia A. As shown here, exophytic hematomas in the oral cavity can be safely removed and nasotracheal intubation with fiberoptic bronchoscopy may be useful in patients with coagulopathy disease.

Download Full-text

Successful Management of Acquired Hemophilia A Associated with Bullous Pemphigoid: A Case Report and Review of the Literature

Case Reports in Hematology ◽

10.1155/2017/2057019 ◽

2017 ◽

Vol 2017 ◽

pp. 1-7 ◽

Cited By ~ 4

Author(s):

Quentin Binet ◽

Catherine Lambert ◽

Laurine Sacré ◽

Stéphane Eeckhoudt ◽

Cedric Hermans

Keyword(s):

Bullous Pemphigoid ◽

Neutralizing Antibodies ◽

Hemophilia A ◽

Rare Condition ◽

Term Treatment ◽

Acquired Hemophilia A ◽

Acquired Hemophilia ◽

Short Term Treatment ◽

Life Threatening ◽

Dose Levels

Background. Acquired hemophilia A (AHA) is a rare condition, due to the spontaneous formation of neutralizing antibodies against endogenous factor VIII. About half the cases are associated with pregnancy, postpartum, autoimmune diseases, malignancies, or adverse drug reactions. Symptoms include severe and unexpected bleeding that may prove life-threatening.Case Study. We report a case of AHA associated with bullous pemphigoid (BP), a chronic, autoimmune, subepidermal, blistering skin disease. To our knowledge, this is the 25th documented case of such an association. Following treatment for less than 3 months consisting of methylprednisolone at decreasing dose levels along with four courses of rituximab (monoclonal antibody directed against the CD20 protein), AHA was completely cured and BP well-controlled.Conclusions. This report illustrates a rare association of AHA and BP, supporting the possibility of eradicating the inhibitor with a well-conducted short-term treatment.

Download Full-text