title: Acquired factor XIII deficiency: a therapeutic challenge.
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.
authors.affiliation: Division of Angiology and Haemostasis, University Hospitals of Geneva and Faculty of Medicine of Geneva, 1211 Geneva 14, Switzerland.
authors.forename: Françoise
authors.initials: F
authors.lastname: Boehlen
authors.forename: Alessandro
authors.initials: A
authors.lastname: Casini
authors.forename: Carlo
authors.initials: C
authors.lastname: Chizzolini
authors.forename: Behrouz
authors.initials: B
authors.lastname: Mansouri
authors.forename: Hans Peter
authors.initials: HP
authors.lastname: Kohler
authors.forename: Verena
authors.initials: V
authors.lastname: Schroeder
authors.forename: Guido
authors.initials: G
authors.lastname: Reber
authors.forename: Philippe
authors.initials: P
authors.lastname: de Moerloose
mesh_terms: D000368:Aged; D000465:Algorithms; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use; D005176:Factor XIII / Q000037:antagonists & inhibitors / Q000096:biosynthesis*; D005177:Factor XIII Deficiency / Q000628:therapy*; D005341:Fibrinolysin / Q000627:therapeutic use; D006405:Hematology / Q000379:methods; D006470:Hemorrhage / Q000139:chemically induced* / Q000628:therapy*; D006801:Humans; D007136:Immunoglobulins / Q000627:therapeutic use; D007163:Immunosorbent Techniques; D008297:Male; D011241:Prednisone / Q000627:therapeutic use; D000069283:Rituximab; D013997:Time Factors; D054556:Venous Thromboembolism / Q000150:complications / Q000628:therapy
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D007136:Immunoglobulins; D000069283:Rituximab; D003520:Cyclophosphamide; D005176:Factor XIII; D005341:Fibrinolysin; D011241:Prednisone