Factor V Deficiency in Korean Patients: Clinical and Laboratory Features, Treatment, and Outcome

Abstract

Due to rarity of factor V (FV) deficiency, there have been only a few case reports in Korea. We retrospectively analysed the clinical-laboratory features of FV deficiency in 10 Korean patients. Between January 1987 and December 2013, 10 case reports published in a Korean journal or proceedings of Korea Society on Thrombosis and Hemostasis were reviewed. Severity is defined as mild (> 5% of factor activity), moderate (1%–5%), and severe (< 1%). The median age at diagnosis, six males and four females, was 26 years (range, 1 month-73 years). Six of 10 patients were classified as moderate, three as mild, and one as severe disease. Eight patients were diagnosed as inherited FV deficiency. The most frequent symptoms were mucosal tract bleedings (40%) such as epistaxis, and menorrhagia in female. Hemarthroses and postoperative bleeding occurred in one and four patients, respectively. Life-threatening bleeding episodes occurred in the peritoneal cavity (n = 2), central nerve system (n = 1), and retroperitoneal space (n = 1). No lethal haemorrhages happened to patients with mild disease. The majority of bleeding episodes were controlled with local measures and fresh-frozen plasma replacement. Two acquired FV deficient-patients showing life-threatening haemorrhages received the immunosuppressive therapy, but one of them died from postoperative bleeding complications. Despite the small sample size of this study due to rarity of the disease, we found that Korean patients with FV deficiency had similar clinical manifestations and treatment outcomes shown in previous studies.

Successful coronary stenting in a patient with factor V deficiency in the absence of fresh frozen plasma transfusion

“…Although F5D is defined as a mild and severe disease in cases with activity >5% and <1%, respectively, factor V activity does not necessarily predict bleeding severity or clinical features, and many patients may present with a mild disease even with factor V activity of <1%. [ 2 ] However, F5D therapy depends on the factor V activity level, and is classified as bleeding control and the removal of inhibitors or antibodies of factor V. FFP or platelet transfusion is recommended to control bleeding. In particular, transfusing FFP is recommended to maintain factor V activity at >25% to 30% in cases of bleeding, invasive testing, or surgery.…”

“…This strategy was based on previous reports in which factor V activity was not related to bleeding severity. [ 2 , 6 ] Moreover, successful childbirth delivery was possible without an FFP transfusion, and the success rate for bleeding control was low with FFP transfusion. [ 3 , 6 ] Fortunately, mucosal bleeding in the present case was controlled by adjusting the antithrombotic drug dose.…”

“…Factor V deficiency (F5D) is a rare hematological disorder with an estimated incidence of 1 case per million people. [ 1 , 2 ] Until now, more than 200 cases have been recorded worldwide in the literature. [ 2 ] F5D patients present with various clinical manifestations.…”

“…[ 1 , 2 ] Until now, more than 200 cases have been recorded worldwide in the literature. [ 2 ] F5D patients present with various clinical manifestations. Although mucosal bleeding is the most common, fatal bleeding complications are also possible.…”

“…Most studies recommend preinterventional or preoperative supplementation with fresh frozen plasma (FFP) to reduce bleeding risk. [ 2 , 3 ] However, in addition to the bleeding risk caused by antithrombotic therapy, the hypercoagulable state in coronary intervention has an adverse effect on stent thrombosis, mortality, and prognosis during the postinterventional period. [ 4 ] The contemporary standard therapy for significant coronary artery stenosis is implanting a drug-eluting stent (DES).…”