[Severe hemorrhagic syndrome in a high responder patient with hemophilia A. Value of porcine factor VIII].
The case is reported of a high responder haemophiliac A, aged 37 yr, who had a severe bleeding episode after surgical treatment of a renal stone. Preoperative anti-factor VIII antibody concentration was 2 UB. Although the surgery was carried out with no real problem (partial nephrotomy), on the 5th postoperative day, there was a sudden increase in the concentration of anti-factor VIII antibodies (21 UB) with a concomitant drastic fall in Factor VIII C level, and important bleeding at the surgical site. The sequential administration of human Factor VIII, "activated prothrombin complex concentrates" (FEIBA followed by AUTOPLEX), and total nephrectomy failed to stem the haemorrhage. Despite intravenous gammaglobulins, the anti-factor VIII titre reached 1300 UB. Bleeding could only be controlled by concentrated porcine Factor VIII (HYATE-C), 100 U.kg-1.8 h-1. This treatment was continued for 50 days, with no side-effects. An average dose of 130 U.kg-1.8 h-1 maintained a residual level of Factor VIII between 1 and 23%. There was a simultaneous fall in the antibody-concentration to less than 1 UB, due to steroid treatment.
Boullet
M
M
Département d'Anesthésie Réanimation Chirurgicale II, Hôpital Régional, Centre Hospitalier Universitaire, Lille.
Goudemand
J
J
Smith
P
P
Willot
I
I
Ballestrazzi
V
V
D000328:Adult; D000818:Animals; D003029:Coagulants / Q000008:administration & dosage; D015949:Factor IXa; D005169:Factor VIII / Q000008:administration & dosage* / Q000032:analysis / Q000037:antagonists & inhibitors / Q000276:immunology; D005938:Glucocorticoids / Q000627:therapeutic use; D006467:Hemophilia A / Q000150:complications* / Q000276:immunology; D006470:Hemorrhage / Q000209:etiology* / Q000628:therapy; D006801:Humans; D008297:Male; D011183:Postoperative Complications / Q000209:etiology* / Q000628:therapy; D012697:Serine Endopeptidases / Q000008:administration & dosage; D013552:Swine
D003029:Coagulants; D005938:Glucocorticoids; D005169:Factor VIII; D012697:Serine Endopeptidases; D015949:Factor IXa
Syndrome hémorragique sévère chez un hémophile A bon répondeur. Intérêt du facteur VIII porcin.