OBJECTIVE AND IMPORTANCE: Acquired haemophilia is a rare hemorrhagic disease caused by inhibitory autoantibodies against coagulation factor VIII. Rituximab has become a popular choice for immunosuppressive therapy in acquired haemophilia, almost with the same schedule of 375 mg/m(2) per week for 4-6 doses. While the effect of low-dose rituximab has seldom been reported. CLINICAL PRESENTATION: We report a patient, aged 88 years, who developed acquired haemophilia with severe hemorrhage and elevation of carbohydrate antigen 125 (CA125), but in the absence of a detectable cause. INTERVENTION: We prescribed a low-dose rituximab alone (100 mg per week for a total of four infusions) for the patient, different from the conventional usage, but received a similar effect. In addition, the patient was diagnosed with immune thrombocytopenia 22 months after rituximab, while FVIII activity and activated partial thromboplastin time remained within the normal range. After four infusions of low-dose rituximab, the platelet count recovered. CONCLUSION: At a follow-up of 34 months, the patient remains in remission without further treatment, suggesting low-dose rituximab seems to be a safe and effective regimen for the elderly patients with acquired haemophilia.
OBJECTIVE AND IMPORTANCE: Acquired haemophilia is a rare hemorrhagic disease caused by inhibitory autoantibodies against coagulation factor VIII. Rituximab has become a popular choice for immunosuppressive therapy in acquired haemophilia, almost with the same schedule of 375 mg/m(2) per week for 4-6 doses. While the effect of low-dose rituximab has seldom been reported. CLINICAL PRESENTATION: We report a patient, aged 88 years, who developed acquired haemophilia with severe hemorrhage and elevation of carbohydrate antigen 125 (CA125), but in the absence of a detectable cause. INTERVENTION: We prescribed a low-dose rituximab alone (100 mg per week for a total of four infusions) for the patient, different from the conventional usage, but received a similar effect. In addition, the patient was diagnosed with immune thrombocytopenia 22 months after rituximab, while FVIII activity and activated partial thromboplastin time remained within the normal range. After four infusions of low-dose rituximab, the platelet count recovered. CONCLUSION: At a follow-up of 34 months, the patient remains in remission without further treatment, suggesting low-dose rituximab seems to be a safe and effective regimen for the elderly patients with acquired haemophilia.
Entities:
Keywords:
Acquired haemophilia; Factor VIII inhibitor; Rituximab
Authors: Jennifer Nardella; Domenico Comitangelo; Renato Marino; Giuseppe Malcangi; Marco Damiano Barratta; Carlo Sabba; Antonio Perrone Journal: J Med Cases Date: 2022-04-12
Authors: Andreas Tiede; Peter Collins; Paul Knoebl; Jerome Teitel; Craig Kessler; Midori Shima; Giovanni Di Minno; Roseline d'Oiron; Peter Salaj; Victor Jiménez-Yuste; Angela Huth-Kühne; Paul Giangrande Journal: Haematologica Date: 2020-05-07 Impact factor: 9.941