Lack of bleeding in patients with severe factor VII deficiency

Abstract

Factor VII deficiency, although rare, is now recognized as the most common autosomal recessive inherited factor deficiency. It is usually considered to be associated with bleeding only in the severely affected subject and heterozygotes (>10%) are not considered at risk. The general recommendation for surgery is to achieve a FVII level in excess of 15% (0.15 1U/mL). We present three cases of severe factor VII deficiency, each of whom appeared hemostatically competent based on clinical history. Subject 1 is a 33 year-old African-American female with a baseline FVII of <1%, who had a fractured tibia requiring open reduction with internal fixation without any FVII replacement and subsequently underwent successful laparoscopic knee surgery with a factor VII level measured at 6%. Subject 2 is a 58 year-old African-American female with a factor VII level of 9% who underwent an elective left total hip replacement without any factor replacement and had no excessive bleeding, but who sustained a pulmonary embolism postoperatively. Subject 3 is a 19-year-old African-American male with a baseline FVII of 1% with a history of active participation in football without noticeable injury and who underwent an emergent appendectomy without bleeding. These three cases represent individuals with the severe form of FVII deficiency who did not exhibit excessive bleeding when challenged with surgical procedures. The clinical history would appear the most valuable tool in predicting the likelihood of bleeding in these patients, and we suggest that the presumption that all patients with severe FVII deficiency should receive replacement therapy before surgical procedures may not be valid in all cases. Am.

Congenital FVII deficiency and thrombotic events after replacement therapy

“…Patients with this mutation (Arg304Gln) are asymptomatic in general or only paucisymptomatic and seldom need substitution therapy. This aspect has been emphasized by several Authors [13,14,30] and is fully shared by the Padua group [15,31]. Finally, the study report underscore once again the need for an accurate diagnosis of the type of FVII defect using thromboplastins of different origin [7,15].…”

“…FVII antigen is normal [7]. After our first description several other cases have been reported in different countries [11][12][13][14]. Bleeding tendency was always reported to be mild or absent.…”

Low levels of activated factor VII in systemic sclerosis

“…These preliminary observations may have additional clinically meaningful implications. At variance with congenital deficiencies, patients with severe acquired factor VII deficiency appear hemostatically competent and do not exhibit a bleeding tendency [37]. On the contrary, increased FVIIa consumption, leading to low FVIIa activity in plasma, has been associated with an enhanced predisposition to develop severe arterial thrombosis [38] and acquired factor VII deficiency due to isolated consumption or proteolytic degradation by leucocyte proteases are frequently observed in patients with severe systemic sepsis [39].…”

Prevention of bleeding and hemorrhagic complications in surgical patients with inherited factor VII deficiency

“…Apart from the early mobilization of patients, on postoperative day 1, no other antithrombotic prophylaxis was introduced. Literature data show that FVII level of more than 25% is sufficient to secure proper hemostasis during the perioperative period in patients with inherited FVII deficiency [1,2,4]. In some publications, rFVIIa is recommended every 3-4 h on postoperative day 1 and 2 because of a short -ca.…”