Management of Arthropathy in Moderately Deficient Factor VII Patients: Results From Hospital Developed Treatment Protocol

Abstract

Background: Factor VII deficiency (FVIId) is a rare inherited bleeding disorder whose prevalence is estimated to be 1 in 300,000 - 500,000 in general population. In India, the epidemiological data on factor VII (FVII) disorder are scarce. Serious arthropathy is observed in cases which are severely affected with spontaneous bleeding and hemarthrosis. In such cases, orthopedic surgery is required. Fresh frozen plasma, prothrombin complex concentrates, factor VII concentrates - plasma derived and recombinant factor VII (rFVII) are different types of preparations which are available as therapeutic options in treatment for bleeding occurring during or after surgery in FVIId patients. The main objective of our study was to give a result of the treatment protocol for FVIId patients undergoing orthopedic surgery in our hospital and compare its results with other studies conducted around the world.

Methods: A retrospective study on patients admitted during 2008 - 2014 was carried out with 10 patients diagnosed with FVIID, of whom six undergone surgery at Kasturba Hospital, Manipal, west coastal region of southern India.

Results: FVII baseline plasma levels were less than 10 IU/dL in our patients. Three patients underwent complete hip replacement, while rest of the three patients underwent various other arthroscopic procedures. rFVII was administered every 8 hours on the surgery day, followed by ever 12 - 24 hours for subsequent days in hospital depending on the type of surgery that the patient underwent. FVII levels in plasma were determined once before and once after the surgery, but the dosing of rFVII was not dependent on it. Dosing of rFVII on the day of surgery was 10 - 25 ug/kg and on following days, it was 15 - 30 ug/kg. The number of dosing on patients ranged from 16 to 31. None of our patients developed serious bleeding episode, thus no blood transfusion was required.

Conclusion: Our study results show that when rFVII is administered according to our treatment regimen, it is effective for patients with arthropathy. It also proves that rFVII is the best hemostatic agent for FVII patients.




J Hematol. 2016;5(3):94-98
doi: http://dx.doi.org/10.14740/jh296e