Portal vein thrombosis in a patient with severe haemophilia A and F V G1691A mutation during continuous infusion of F VIII after intramural jejunal bleeding – Successful thrombolysis under heparin therapy

摘要: We report on a 14-year-old boy with severe haemophilia A who developed portal vein thrombosis during continuous infusion of F VIII. For treatment posttraumatic intramural jejunal haematoma extension into the mesenterium patient received (CI) high purity VIII concentrate, starting an initial bolus injection 100 IU VIII/kg bw and followed by 4–5 bw/h i.v. plasma activity ranged between 47 88%. Resorption was proven abdominal ultrasonic follow-ups. After 3 weeks CI thrombus formation in detected ultrasound confirmed duplex ultrasound. Subsequent to diagnosis heparinised unfractionated heparin (UFH 300–450 IU/kg/d i.v.). In order induce further resorption haematoma, concentrate given concomitantly (50 IU/kg twice daily) phase treatment. 14 days anticoagulant therapy UFH, regimen changed low molecular weight (LMWH; Fraxiparin 0.3™; 2850 anti-X activity/d s.c.; 60 kg). dosage gradually reduced advanced thereafter switched prophylaxis (40 times weekly). Complete lysis observed after 6 months UFH LMWH respectively without any complications. Thereafter discontinued. Thrombophilic screening revealed no abnormalities except heterozygous V G1691A.