Life-threatening bleeding in a patient with pemphigoid-induced acquired hemophilia A and successfully treated with rituximab and rFVIIa: A case report.

Hongbing Ma, Hong Chang

Medicine 2021 Jan 22

filter terms:

Acquired hemophilia A (AHA) is a rare bleeding disorder with prolonged activated partial thromboplastin time (aPTT). Severe hemorrhage may occur, especially in refractory AHA. We reported a 63-year-old man who suffered from life-threatening bleeding after the onset in lower limbs. The patient was diagnosed as AHA which was related to pemphigoid. The patient had no response to the first-line treatment with corticosteroid and cyclophosphamide. Meanwhile, fatal hemorrhage occurred successively in thoracic cavity and right frontal lobe. rFVIIa and rituximab were administered. The patient survived from the life-threatening hemorrhage with a normal aPTT. His aPTT and FVIII:C level was normal during the follow-up of 6 months. Rituximab and rFVIIa can play a critical role in rescuing AHA that is refractory to the first-line treatment. Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.

Citation

Hongbing Ma, Hong Chang. Life-threatening bleeding in a patient with pemphigoid-induced acquired hemophilia A and successfully treated with rituximab and rFVIIa: A case report. Medicine. 2021 Jan 22;100(3):e24025