P80 | Early rituximab in acquired haemophilia A: two cases report
Year: 2025
Type: article
Study design: Case Report
Abstract: Background: Acquired hemophilia A (AHA) is a rare event caused by the development of autoantibodies against factor VIII protein (FVIII). Guidelines recommend rituximab or cytotoxic agents as second-line therapy after 3-4 weeks of corticosteroids to eradicate FVIII inhibitor. We present two case reports of early rituximab use in AHA patients.Case 1: A man with myelomonocytic leukemia presented with widespread hematomas. Lab results showed anemia, prolonged aPTT, FVIII activity 0%, and an inhibitor level of 1.9 BU. Initial treatment with Methylprednisolone, APCC and rVIIa had limited response. Early rituximab therapy (375 mg/m2 weekly for 4 weeks) led to progressive hematoma resolution and eliminated the need for transfusions. After 4 weeks, aPTT was 35s, FVIII activity increased to 62%, and the inhibitor level dropped to 0.4 BU. Case 2: A man with metastatic gastric cancer in chemotherapy presented with left gluteal and abdominal swelling, severe anemia, and prolonged aPTT, reduced FVIII levels and the presence of FVIII inhibitor. CT scan showed the presence of muscular hematomas requiring embolization of the left gluteal and superficial left circumflex iliac arteries. Methylprednisolone and rVIIa were initiated with stability for two weeks. New hematomas prompted rituximab therapy, resulting in hematoma resolution, clinical improvement, normalizing aPTT, FVIII activity with FVIII inhibitor complete eradication.Conclusions: Early rituximab use, contrary to guidelines, effectively restored FVIII levels, reduced inhibitors, and resolved hemorrhagic symptoms in AHA patients.
Source: Italian Journal of Medicine
Authors: Vo Van Kha, Tran Thi Huong Ly, Pham Thi Thanh Hoa, Vo Huynh Nhu, Damas Hendriansyah, Nusratuddin Abdullah, Rudy B. Leonardy, Mardiah Tahir, Samrichard Rambulangi, Firdaus Hamid, Filomena Pietrantonio, Vera Kopsaj, Francesco Rosiello, Aurora Tomassoni, Luca Moriconi, Donatella Padua, Kiki Rizki Amelia, Efendi Lukas, Nurbani Bangsawan, Isharyah Sunarno, Retno Budiarti Farid, Sriwijaya Qadar, Rabab H. Elshaikh, Ashok Kumar Sah, Abdelrahman Mohamed Abukanna, Abdulrahman Algarni, Ammar Abdelmola, Hussam Ali Osman, Tagwa Yousif Elsayed Yousif, Elryah I. Ali, Hiba Ali, Ayman Hussein Alfeel, Thanh Long Nguyen, Victor Storm, Lam Toi Phung, Cong Thien Le, Thi Thu Ha Le, Thi Hoa Nguyen, Son Tung Vu, Thien Thang Tran, Minh Hanh Vu, Van Tuan Nguyen, Francesco Ventrella, Anna Belfiore, Antonio Greco, Franco Mastroianni, Salvatore Lenti, Domenico Ruggiero, Michele Cannone, Vincenzo Ostilio Palmieri, Massimo Errico, Giuseppe Mancia, Guido Grassi, Sverre E. Kjeldsen
Language: English
Subfield: Hematology
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W4413656042