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

doi:10.1160/TH12-08-0604

Thrombosis and Haemostasis, Inhaltsverzeichnis

Thromb Haemost 2013; 109(03): 479-487
DOI: 10.1160/TH12-08-0604

Blood Coagulation, Fibrinolysis and Cellular Haemostasis

Schattauer GmbH

Acquired factor XIII deficiency: a therapeutic challenge

Françoise Boehlen

¹Angiology and Haemostasis Division, University Hospitals and School of Medicine, Geneva, Switzerland

,

Alessandro Casini

¹Angiology and Haemostasis Division, University Hospitals and School of Medicine, Geneva, Switzerland

,

Carlo Chizzolini

²Immunology& Allergy, University Hospitals and School of Medicine, Geneva, Switzerland

,

Behrouz Mansouri

³Division of Transfusion Medicine, University Hospital, Bern, Switzerland

,

Hans Peter Kohler

⁴Laboratory for Haemostasis Research, Department of Haematology, University Hospital of Bern and Department of Internal Medicine, Spital Netz Bern Hospitals, Bern, Switzerland

,

Verena Schroeder

⁴Laboratory for Haemostasis Research, Department of Haematology, University Hospital of Bern and Department of Internal Medicine, Spital Netz Bern Hospitals, Bern, Switzerland

,

Guido Reber

¹Angiology and Haemostasis Division, University Hospitals and School of Medicine, Geneva, Switzerland

,

Philippe de Moerloose

¹Angiology and Haemostasis Division, University Hospitals and School of Medicine, Geneva, Switzerland

› Institutsangaben

Abstract

Summary

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.

Keywords

Acquired factor XIII deficiency - factor XIII concentrates - thrombosis - immunosuppression - Fibrogammin P^®

Volltext

Referenzen

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