Acquired factor XIII deficiency: a therapeutic challenge

Boehlen, Françoise; Casini, Alessandro; Chizzolini, Carlo; Mansouri, Behrouz; Kohler, Hans Peter; Schroeder, Verena; Reber, Guido; de Moerloose, Philippe (2013). Acquired factor XIII deficiency: a therapeutic challenge. Thrombosis and haemostasis, 109(3), pp. 479-487. Stuttgart: Schattauer 10.1160/TH12-08-0604

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

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