Acquired factor XIII deficiency: a therapeutic challenge.

Françoise Boehlen, Alessandro Casini, Carlo Chizzolini, Behrouz Mansouri, Hans Peter Kohler, Verena Schroeder, Guido Reber, Philippe de Moerloose

Division of Angiology and Haemostasis, University Hospitals of Geneva and Faculty of Medicine of Geneva, 1211 Geneva 14, Switzerland.

Thrombosis and haemostasis 2013 Mar

filter terms:

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.

Citation

Françoise Boehlen, Alessandro Casini, Carlo Chizzolini, Behrouz Mansouri, Hans Peter Kohler, Verena Schroeder, Guido Reber, Philippe de Moerloose. Acquired factor XIII deficiency: a therapeutic challenge. Thrombosis and haemostasis. 2013 Mar;109(3):479-87