Acquired factor XIII deficiency: a therapeutic challenge
Data(s) |
2013
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Resumo |
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. |
Formato |
application/pdf |
Identificador |
http://boris.unibe.ch/13833/1/2013_Acquired%20factor%20XIII%20deficiency_B.%20Mansouri.pdf Boehlen, Françoise; Casini, Alessandro; Chizzolini, Carlo; Mansouri, Behrouz; Kohler, Hans Peter; Schroeder, Verena; Reber, Guido; de Moerloose, Philippe (2013). Acquired factor XIII deficiency: a therapeutic challenge. Thrombosis and haemostasis, 109(3), pp. 479-487. Stuttgart: Schattauer 10.1160/TH12-08-0604 <http://dx.doi.org/10.1160/TH12-08-0604> doi:10.7892/boris.13833 info:doi:10.1160/TH12-08-0604 info:pmid:23306660 urn:issn:0340-6245 |
Idioma(s) |
eng |
Publicador |
Schattauer |
Relação |
http://boris.unibe.ch/13833/ |
Direitos |
info:eu-repo/semantics/restrictedAccess |
Fonte |
Boehlen, Françoise; Casini, Alessandro; Chizzolini, Carlo; Mansouri, Behrouz; Kohler, Hans Peter; Schroeder, Verena; Reber, Guido; de Moerloose, Philippe (2013). Acquired factor XIII deficiency: a therapeutic challenge. Thrombosis and haemostasis, 109(3), pp. 479-487. Stuttgart: Schattauer 10.1160/TH12-08-0604 <http://dx.doi.org/10.1160/TH12-08-0604> |
Palavras-Chave | #610 Medicine & health |
Tipo |
info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion PeerReviewed |