Presentation Details
| Acquired hemophilia secondary to the Transplantation of Hematopoietic Progenitors.A Systematic Review Rafael Pichardo-Rodriguez1, Cristhian Gonzales- Rospigliosi1, Dalia Pimentel-Ramirez1, Aracely Carrasco-Espinoza1, Lara Vela-Rojas1, Diana-Cristina Ramírez-Meyhuay1, Jhony A.De La Cruz-Vargas1, Alfredo Wong Chang2, Cristobal Frutos3. 1Instituto de Investigaciones en Ciencias Biomédicas (INICIB).Universidad Ricardo Palma, Lima, Peru.2Unidad de Trasplante de Médula Ósea.Hospital Nacional Edgardo Rebagliati-Essalud, Lima, Peru.3Instituto de Previsión Social, Asuncion, Paraguay |
Abstract
BACKGROUND: Acquired hemophilia (AH) is a severe bleeding disorder caused by autoantibodies directed against coagulation factor VIII or IX. Hematopoietic stem cell transplantation (HSCT), has been associated with the development of AH though evidence is limited. OBJECTIVES: To describe the clinical characteristics, therapeutic approaches, and outcomes in patients with AH developed after HSCT. METHODS: A systematic review of case reports was conducted following the PRISMA statement (PROSPERO: CRD42024512037). Case reports of post-transplant patients with confirmed AH diagnosis were included. Risk of bias was assessed, and qualitative synthesis of data was performed. RESULTS: Twelve clinical cases were included. The 58% presented high quality. Patients developed AH within a variable post-transplant time range (4 days to >2 years), with clinical presentations including hematomas, systemic hemorrhages, and epistaxis. A variety of pre-transplant conditioning regimens were observed, with thiotepa/cyclophosphamide being the most common. Allogeneic transplantation was more frequent than autologous. The majority of patients developed inhibitors to factor VIII and exhibited reduced levels of FVIII activity. Treatment included prothrombin complex concentrates, corticosteroids, and immunosuppressants, with Rituximab showing significant improvements in some cases. Despite treatment initiation, 50% of patients recovered, 25% had disease relapse and 25% died of hemorrhagic complications. CONCLUSIONS: Post-transplant AH is a severe complication requiring multidisciplinary evaluation and management. Navigating treatment specially in allogeneic HSCT recipients for malignant diseases where graft preservation is crucial poses a unique challenge. Although improvements were observed with different treatments, complications are notable, highlighting the need for further research to guide optimal management of this population.
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