Acquired factor XIII deficiency: a therapeutic challenge

摘要: Less than 60 cases of acquired factor (F)XIII deficiencies have been reported, most having distinct clinical features. To illustrate the therapeutic challenges FXIII inhibitors, we report a case 65-year-old patient with no previous bleeding history who suddenly developed massive haemorrhages associated to strong and isolated inhibitor. No underlying disorder has detected till now after three years follow-up. Despite aggressive treatment prednisone, rituximab, cyclophosphamide, immunoglobulin, immunoadsorption immune tolerance his inhibitor is still present, although at low titre benefit since more bleed one year. Moreover had venous thromboembolic complication. After review management deficiency patients based on haemophilia discuss possible strategy for such difficult cases.