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Acquired factor XIII deficiency: a therapeutic challenge

Mansouri, Behrouz
Kohler, Hans Peter
Schroeder, Verena
Published in Thrombosis and haemostasis. 2013, vol. 109, no. 3, p. 479-487
Abstract 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 AgedAlgorithmsAntibodies, Monoclonal, Murine-Derived/therapeutic useCyclophosphamide/therapeutic useFactor XIII/antagonists & inhibitors/biosynthesisFactor XIII Deficiency/therapyFibrinolysin/therapeutic useHematology/methodsHemorrhage/chemically induced/therapyHumansImmunoglobulins/therapeutic useImmunosorbent TechniquesMalePrednisone/therapeutic useTime FactorsVenous Thromboembolism/complications/therapy
PMID: 23306660
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Research groups Geneva Platelet Group (13)
La matrice extracellulaire (651)
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BOEHLEN, Françoise et al. Acquired factor XIII deficiency: a therapeutic challenge. In: Thrombosis and haemostasis, 2013, vol. 109, n° 3, p. 479-487. doi: 10.1160/TH12-08-0604 https://archive-ouverte.unige.ch/unige:78248

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Deposited on : 2015-12-07

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