• Medicine · Jan 2021

    Case Reports

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

    • Hongbing Ma and Hong Chang.
    • Department of Hematology, West China Hospital, Sichuan University, Chengdu 610041, China.
    • Medicine (Baltimore). 2021 Jan 22; 100 (3): e24025.

    RationaleAcquired hemophilia A (AHA) is a rare bleeding disorder with prolonged activated partial thromboplastin time (aPTT). Severe hemorrhage may occur, especially in refractory AHA.Patient ConcernsWe reported a 63-year-old man who suffered from life-threatening bleeding after the onset in lower limbs.DiagnosesThe patient was diagnosed as AHA which was related to pemphigoid.InterventionsThe 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.OutcomesThe 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.LessonsRituximab 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.

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