Gala Cabirta-Touzon, Servicio de Hematología y Hemoterapia, Hospital Universitario Álvaro Cunqueiro, EOXI Vigo, Instituto de Investigación Sanitaria Galicia-Sur (IISGS), Vigo, España
Irene Castaño-Caballero, Servicio de Hematología y Hemoterapia, Hospital Universitario Álvaro Cunqueiro, EOXI Vigo, Instituto de Investigacion Sanitaria Galicia-SUR, Vigo, España
Raquel Iglesias-Varela, Servicio de Hematología y Hemoterapia, Hospital Universitario Álvaro Cunqueiro, EOXI Vigo, Instituto de Investigacion Sanitaria Galicia-SUR, Vigo, España
Manuel Rodríguez-López, Servicio de Hematología y Hemoterapia, Hospital Universitario Álvaro Cunqueiro, Instituto de Investigación Sanitaria Galicia Sur, EOXI Vigo, Vigo, España
Introduction: The development of inhibitory antibodies against factor VIII (FVIII) is a complication in approximately 30% of patients with severe hemophilia A, increasing bleeding frequency and resistance to treatment. Objective: To report the experience of a tertiary care center in the management of an inhibitor or neutralizing antibody against FVIII in a patient with mild hemophilia A and to provide a brief critical review of the topic. Material and methods: The identified mutation was c.6301 C > G in exon 22 of the F8 gene, not among the missense variants previously associated with the highest inhibitor risk. In the absence of DDAVP response, the acute bleeding episode was managed with recombinant factor VIIa, and immunosuppression with prednisone was initiated. On day 7, as the inhibitor titre rose to 17 BU, rituximab (anti-CD20) was added at the standard dose of 375 mg/m² weekly for four weeks. Results: The inhibitor became undetectable at 2.5 months, and baseline FVIII: C levels were restored at 3.5 months, with a positive DDAVP challenge. At 12 months, the patient remains in complete remission. Conclusions: This case highlights the heterogeneity in managing inhibitors in non-severe haemophilia A, the lack of standardised guidelines, and the need for an individualised therapeutic approach. The complementary role of emicizumab is discussed, along with modifiable risk factors — FVIII dose, treatment duration, and surgical exposure — for inhibitor prevention.
Keywords: Non-severe haemophilia A. Inhibitors AntiFVIII. Immunosuppression. Erradication.