Introduction: Bleeding from esophageal varices is a serious clinical condition in hemophilia patients due to congenital deficiency
or lack of clotting factors VIII (in hemophilia A) and IX (in hemophilia B), decreased clotting factor II, VII, IX, X synthesis
in the course of chronic liver disease and hipersplenic thrombocytopenia. The aim of this study was to assess the efficacy and
safety of endoscopic sclerotherapy in acute esophageal variceal bleeding and in secondary prophylaxis of hemorrhage. The
aim was also to investigate the optimal activity of deficiency factors VIII or IX and duration of replacement therapy required
to ensure proper hemostasis after sclerotherapy procedures.
Material and methods: 22 hemophilia patients (A-19, B-4) with coexistent liver cirrhosis and active esophageal variceal bleeding
treated with endoscopic sclerotherapy were subjected to prospective analysis. The patients who survived were qualified
to repeated sclerotherapy procedures every 3 weeks within secondary prophylaxis of bleeding (investigated group).
A 3-day substitution therapy enhanced the infusion of the deficient or lacking factor in doses allowing to reach 80-100% of
normal value activity of factor VIII on the 1st day and 60-80% in the next two days. The desired activity of factor IX was 60-
80% and 40-60% respectively. The control group consisted of 20 non-hemophiliac patients with liver cirrhosis comparable in
terms of age, sex, stage of advancement of liver cirrhosis, who underwent the same medical proceedings as the investigated
group.
Results: Active esophageal bleeding was stopped in 21 of 22 (95%) hemophilia patients. Complications were observed in 3 patients;
2 patients died. The rate of hemostasis, complications and deaths in the control group were comparable and no statistical
differences were found. In hemophilia patients subjected to secondary prophylaxis of hemorrhage, in 18 of 20 (80%),
complete eradication of esophageal varices was achieved after 4 to 7 sclerotherapy procedures in 1 patient (average 5.4). Recurrent
bleeding was observed in 15% of patients, complication in 20%; 1 patient died. Time lapse from bleeding to eradication
was 12-21 weeks (average 15.2).
In the control group the rate of variceal eradication, complication and deaths was comparable and no statistical differences
were found. The usage of factor VIII concentrates was as follows: in hemophilia A, in a severe form - 80.9 U/kg b.w./day, in hemophilia
A in a severe form with an inhibitor <5 BU – 95.2 U/kg b.w./day, in mild form – 64.2 U/kg b.w./day and in severe hemophilia
B – 91.6 U/kg b.w./day. Conclusions: Sclerotherapy is an effective method in the management of esophageal variceal
bleeding in hemophilia patients. It is also effective for total eradication of varices when applied as a secondary prophylaxis
of hemorrhage. In our opinion, a 3-day replacement therapy at the applied doses is sufficient to ensure hemostasis and avoid
bleeding complications.
Covered TIPS for secondary prophylaxis of variceal bleeding in liver cirrhosis
