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

• Published in: European journal of pediatrics 1999-12, Vol.158 Suppl 3 (S3), p.S180-S182
• Main Authors: Ettingshausen, C E, Saguer, I M, Kreuz, W
• Format: Article
• Language: English
• Subjects: Adolescent; Anticoagulants - therapeutic use; Coagulants - adverse effects; Factor V - genetics; Factor VIII - adverse effects; Gastrointestinal Hemorrhage - drug therapy; Hemophilia A - complications; Heparin - therapeutic use; Humans; Infusions, Intravenous - adverse effects; Jejunal Diseases - drug therapy; Male; Portal Vein; Thrombophilia - complications; Venous Thrombosis - complications; Venous Thrombosis - etiology
• ISSN: 0340-6199; 1432-1076
• DOI: 10.1007/PL00014351

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