A CASE OF SEVERE HEMOPHILIA-A UNDERGOING SPLENECTOMY FOR IDIOPATHIC PORTAL HYPERTENSION

Since highly purified factor VIII concentrate has been developed as a hemostatic regimen for hemophilia, it keeps on prolonging a life of the hemophiliacs, and increasing a surgical opportunity for them. Though we can see only a few reports of laparotomy for hemophiliacs, laparotomy can be done safely under an appropriate replacement therapy with the factor VIII concentrate. In this paper we report a successful splenectomy for a patient with severe hemophilia presenting with an esophageal varix for idiopathic portal hypertension under the replacement therapy with factor VIII concentrate. The splenectomy was performed to get an effective sclerotherapy and to increase platelet and white blood cell. Post-operative course was uneventful.