RESUMO
UNLABELLED: Surgery for malignant neoplasms in hemophilia patients is no different from standard procedures in the general population on condition that the normal hemostatic activity of deficient factors: VIII in hemophilia A and IX in hemophilia B in perioperative period is ensured. The aim of the study was to determine the type and frequency of malignant neoplasms in hemophilia patients as well as to provide a strategy for establishing safe hemostatic activity in surgically treated hemophilia patients. MATERIAL AND METHODS: In the period 2003-2008, surgical procedures were performed on 19 hemophilia A and B patients with diagnosed malignant neoplasms of various location. The following cases were diagnosed: 9 colorectal neoplasms, 2 pancreatic carcinomas, 2 larynx carcinomas, 1 stomach carcinoma, 1 liver, 1 nasopharyngeal, 1 testicle, 1 prostate and 1 skin. Seventeen patients were hemophilia A (6 severe, 4 severe with inhibitor, including 2 high titre and 2 low titre, 1 moderate and 6 mild) and 2 were hemophilia B (1 severe, 1 moderate). Patients mean age was 55.8 years (22-82). In factor-replacement therapy for patients with no inhibitor the strategy was to maintain the activity of the deficient clotting factor VIII before the operation at 80-100% of normal value, within the 80-100% range on the 1-3 day following surgery, at 60-80% on days 4-6, at 30-60% on days 7-10 day and at 20-40% on all subsequent days until the surgical wound healed. In hemophilia B patients the levels were about 20% lower. Deficient factor was injected every 8 or 12 hours or administered in continuous intravenous infusion. In hemophilia A patients with high titre inhibitors to factor VIII (above 5 Bethesda units/ml) activated prothrombin complex concentrates (aPCC)-FEIBA (Baxter AG) were used at 50-100 U/kg b.m., every 8-12 hours. RESULTS: The nineteen patients with diagnosed malignant neoplasms (in the period 2003-2008) constituted 0.77% of the overall 2475 hemophilia patients entered into the National Registry Congenital of Hemorrhagic Diathesis. An overall number of 26 surgical procedures were performed in this group of 19 hemophilia patients including 20 procedures for malignant neoplasms and 6 for postoperative complications. All patients survived surgery. Two patients with pancreatic carcinoma died in the postoperative period due to multi-organ failure. Complications occured in 7 (37%) patients including 6 (32%) with bleeding complications: haemopneumothorax (n=1), intraperitoneal bleeding (n=2), abdominal parietal hematoma (n=1), hematuria (n=1), bleeding from esophago-pharyngo-cutaneous fistula following total laryngectomy (n=1). Other complications included: ileus (n=1), leakage of pancreato-jejunal anastomosis (n=1), dehiscence of abdominal wound (n=1) and bleeding from duodenal ulcer (n=1). These complications were successfully treated with surgery or endoscopy. CONCLUSIONS: Surgery of malignant neoplasms in hemophilia patients is burdened with a high risk of complications which include bleedings despite adequate replacement therapy and administration of factor eight by-passing concentrates in patients with high titre inhibitor. Therefore surgical procedures involving these patients should be performed in specialized centers with experienced team (surgeon, anaesthesiologist, haematologist) and supported by a laboratory for coagulation disorders.
