Acquired factor XIII deficiency: a therapeutic challenge

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

[img] Text
2013_Acquired factor XIII deficiency_B. Mansouri.pdf - Published Version
Restricted to registered users only
Available under License Publisher holds Copyright.

Download (212kB) | Request a copy

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.

Item Type:

Journal Article (Original Article)

Division/Institute:

?? DCD5A442BF3AE17DE0405C82790C4DE2 ??
04 Faculty of Medicine > Department of Haematology, Oncology, Infectious Diseases, Laboratory Medicine and Hospital Pharmacy (DOLS) > Clinic of Haematology and Central Haematological Laboratory

UniBE Contributor:

Mansouri Taleghani, Behrouz; Kohler, Hans-Peter and Schröder, Verena

Subjects:

600 Technology > 610 Medicine & health

ISSN:

0340-6245

Publisher:

Schattauer

Language:

English

Submitter:

Factscience Import

Date Deposited:

04 Oct 2013 14:35

Last Modified:

29 Oct 2019 13:02

Publisher DOI:

10.1160/TH12-08-0604

PubMed ID:

23306660

Web of Science ID:

000316164200017

BORIS DOI:

10.7892/boris.13833

URI:

https://boris.unibe.ch/id/eprint/13833 (FactScience: 220499)

Actions (login required)

Edit item Edit item
Provide Feedback