Abstract
Introduction and Objectives: Inhibitors against FVIII can occur in the haemophilic and non-haemophilic patients. Venous access is critical to the treatment of the patients with FVIII inhibitors and the prevention of complications due to bleeding. However, there is no guide to the selection of the most appropriate LTCVADs in these patients. The aim of this study was to investigate the providing hemostasis and the results of the placement of LTVADs in patients with FVIII inhibitors.
Patients and Methods: The results of the LTCVADs placement were investigated in 8 patients (7 males, 1 female) with FVIII inhibitors (a median age of 45 years, range 25-73). FVIII inhibitors were revealed in 6 patients with congenital haemophilia A, in the woman with von Willebrand disease and in the patient with acquired idiopathic haemophilia A. In all patients plasma FVIII activity was below 1%, and inhibitor titers were from 120 to 450 BU/ml. Indications, early and delayed complications, hemostatic therapy and implanted period were evaluated. All LTCVADs were monitored from insertion to removal.
Results. In total, thirteen LTCVADs were placed. The indications for placement of LTCVADs were Immune tolerance therapy (5 patients), hemostatic therapy (2 patients) and chemotherapy in woman with von Willebrand disease and mammary cancer. Central Venous Port System (Bard Access System) was implanted in hemophilia A patient simultaneously wiht the hip replacement surgery. The hemostasis in this patient was provided by recombinant activated coagulation factor VII (rFVIIa) (Coagil VII, Generium, Russia) 120 mcg/kg every 2 hours for 2 weeks. However, when rFVIIa therapy after surgery was stopped and antibiotic therapy continued extensive hematoma on the breast developed and the port system was removed. In 7 patients 12 peripherally inserted central catheters (PICCs) (GROSHONG PICC CATHETER, Bard Access System) were placed. PICCs were inserted into brachial veins. All patients received rFVIIa (120 mcg/kg) before the procedure, and experienced no bleeding complications. After the procedure, one patient had some bleeding and required repeated administration of rFVIIa (90 mcg/kg). Overall mean PICC dwell time was 176 days (median 120 days; range 5-463). One patient died due to gastrointestinal bleed in 5 days. Occlusions occurred in 3 cases on 30, 120 and 300 days after PICC insertion in two patients. Accidental withdrawals occurred in one patient. At all three patients PICCs have been placed repeatedly. The obstruction rate was 30% (1.9 per 1000 PICC-days). No infection or thrombotic complications were observed. Six patients are still using a catheter without apparent problems for more than 4 months. There were no specific problems in using PICC with immune tolerance therapy and chemotherapy.
Conclusion. PICCs appear safe to use in the patients with FVIII inhibitors, with acceptably low rates of infectious or thrombotic complications. Catheter occlusion was the most common complication.
Single administration of the rFVIIa provides safe implantation of the PICCs in patients with FVIII inhibitors. PiCCs placement provides significant economic savings (decrease in rFVIIa requirements).
No relevant conflicts of interest to declare.
Author notes
Asterisk with author names denotes non-ASH members.
