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.
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.


CONCLUSIONS
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.
Ettingshausen
C E
CE
Department of Paediatrics III, Haematology and Oncology, Johann Wolfgang Goethe University Hospital, Frankfurt am Main, Germany. cescuriola@zki.uni-frankfurt.de
Saguer
I M
IM
Kreuz
W
W
D000293:Adolescent; D000925:Anticoagulants / Q000627:therapeutic use; D003029:Coagulants / Q000009:adverse effects; D005165:Factor V / Q000235:genetics*; D005169:Factor VIII / Q000009:adverse effects; D006471:Gastrointestinal Hemorrhage / Q000188:drug therapy; D006467:Hemophilia A / Q000150:complications*; D006493:Heparin / Q000627:therapeutic use; D006801:Humans; D007262:Infusions, Intravenous / Q000009:adverse effects; D007579:Jejunal Diseases / Q000188:drug therapy; D008297:Male; D011169:Portal Vein; D019851:Thrombophilia / Q000150:complications*; D020246:Venous Thrombosis / Q000150:complications* / Q000209:etiology
D000925:Anticoagulants; D003029:Coagulants; D005165:Factor V; D005169:Factor VIII; D006493:Heparin