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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
1   Angiology and Haemostasis Division, University Hospitals and School of Medicine, Geneva, Switzerland
,
Alessandro Casini
1   Angiology and Haemostasis Division, University Hospitals and School of Medicine, Geneva, Switzerland
,
Carlo Chizzolini
2   Immunology& Allergy, University Hospitals and School of Medicine, Geneva, Switzerland
,
Behrouz Mansouri
3   Division of Transfusion Medicine, University Hospital, Bern, Switzerland
,
Hans Peter Kohler
4   Laboratory for Haemostasis Research, Department of Haematology, University Hospital of Bern and Department of Internal Medicine, Spital Netz Bern Hospitals, Bern, Switzerland
,
Verena Schroeder
4   Laboratory for Haemostasis Research, Department of Haematology, University Hospital of Bern and Department of Internal Medicine, Spital Netz Bern Hospitals, Bern, Switzerland
,
Guido Reber
1   Angiology and Haemostasis Division, University Hospitals and School of Medicine, Geneva, Switzerland
,
Philippe de Moerloose
1   Angiology and Haemostasis Division, University Hospitals and School of Medicine, Geneva, Switzerland
› Author Affiliations
Further Information

Publication History

Received: 23 August 2012

Accepted after major revision: 07 January 2012

Publication Date:
29 November 2017 (online)

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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®
 

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