warfarin and Factor-XIII-Deficiency

Patients with congenital Factor XIII (FXIII) deficiency have impaired fibrin stabilization and are at high risk for surgical bleeding. Data regarding the use of FXIII concentrates before and during surgery are lacking. The objective of this study was to report the use of plasma-derived FXIII concentrate (Corifact in the United States; Fibrogammin P in other countries) in patients with congenital FXIII deficiency undergoing surgical procedures.. FXIII concentrate at preoperative doses ranging from 25 to 40 U/kg was administered to six patients with congenital FXIII deficiency undergoing major or minor surgeries.. FXIII concentrate was administered immediately before surgery for five surgical cases; three of these patients achieved excellent hemostasis during and after surgery, while two had intraoperative bleeding. In one surgical case, a regular prophylactic dose of FXIII concentrate was administered to the patient 1 week before minor surgery. FXIII concentrate provided rapid replacement of FXIII activity. In all but one of the patients given a dose of FXIII designed to increase FXIII levels more than 50%, there was satisfactory intraoperative and postoperative hemostasis. One patient undergoing aortic valve replacement on cardiopulmonary bypass (CPB) was the exception. Intraoperative bleeding in this patient was associated with lower-than-expected blood levels of FXIII.. Preoperative plasma-derived FXIII concentrate allowed for sufficient hemostasis in most patients with FXIII deficiencies. Additional doses were necessary to achieve hemostasis in one patient who underwent a CPB procedure.

Topics: Adolescent; Adult; Aged; Anticoagulants; Blood Loss, Surgical; Factor XIII; Factor XIII Deficiency; Female; Humans; Male; Middle Aged; Oral Hemorrhage; Postoperative Hemorrhage; Preoperative Care; Thrombosis; Warfarin

A 5-year-old girl in whom the diagnosis of inherited factor XIII deficiency was established at the age of 1 day presented with cryptic tonsillitis along with drowsiness and an abrupt occurrence of getting left interior cross eyed. While an intracranial hemorrhage was expected, cerebral imaging studies surprisingly revealed multiple sino venous thrombosis. In prothrombotic screening studies she and her father were both found to be heterozygous for factor V Leiden mutation along with having elevated levels of lipoprotein(a). Low-molecular-weight heparin was started. Ventriculoperitoneal shunt was applied because of persistence of increased intracranial pressure. Thrombosis disappeared and blood flow was normalized by the end of 2 months and the patient was discharged on coumadin therapy as being well. We would like to report this unusual case and to discuss the possible effects of two major genetic prothrombotic risk factors on inherited bleeding tendency or vice versa.

Topics: Anticoagulants; Cerebral Angiography; Child, Preschool; Consanguinity; Factor V; Factor XIII Deficiency; Female; Heterozygote; Humans; Lipoprotein(a); Sagittal Sinus Thrombosis; Warfarin

Topics: Anti-Infective Agents; Antibodies, Monoclonal; Antibodies, Monoclonal, Murine-Derived; Autoantibodies; Blood Coagulation Factors; Ciprofloxacin; Factor VIIa; Factor XIII; Factor XIII Deficiency; Female; Hemorrhage; Humans; Immunologic Factors; Middle Aged; Recombinant Proteins; Rituximab; Thrombosis; Time Factors; Warfarin