UNIGE document Scientific Article - Case report
previous document  unige:78248  next document
add to browser collection
Title

Acquired factor XIII deficiency: a therapeutic challenge

Authors
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
Identifiers
PMID: 23306660
Full text
Article (Published version) (207 Kb) - document accessible for UNIGE members only Limited access to UNIGE
Structures
Research groups La matrice extracellulaire (651)
Geneva Platelet Group (13)
Citation
(ISO format)
BOEHLEN, Françoise et al. Acquired factor XIII deficiency: a therapeutic challenge. In: Thrombosis and Haemostasis, 2013, vol. 109, n° 3, p. 479-487. https://archive-ouverte.unige.ch/unige:78248

185 hits

0 download

Update

Deposited on : 2015-12-07

Export document
Format :
Citation style :