Background/Aims: Factor V (FV) is a coagulaion factor produced
by liver and stored within platelets. FV deficiency is a rare autosomal
recessive disorder with the incidence of one per 1,000,000 people.
Active FV leads to active Factor X which is an extremely important
coagulation factor to convert prothrombin into thrombin. Hemorrhagic
episodes are commonly expected for FV deficiency however rarely it
could cause thrombotic events.
Materials and Methods: We present the management of a pregnant patient with FV deficiency who developed deep venous thrombosis
when her international normalized ratio (INR) level was 3.
Results: A 30 year-old lady evaluated for increased INR level when she
was 9 weeks pregnant. On her history, this is her first pregnancy and
she had no bleeding history but she had two sisters with FV deficiency.
Her coagulation screen was performed and the prothrombin time and
activated partial thromboplastin time were 34,4 seconds (10.9-14.7)
and 38 seconds (22.5-31.3) respectively. INR level was 3,3 and factor
levels were examined; FV: < %5 (70-120); FVIII: >144,6 (70-150); FX
: 113,3(70-120). She was diagnosed with FV deficiency and followed
every other week at clinic. When she was 16 weeks pregnant, she
was admitted sudden left leg swelling. Work-up revealed deep
vein thrombosis of the distal external iliac, common femoral, deep
femoral and popliteal veins. Treatment with low molecular weight
heparin (LMWH) was done and INR level was checked regularly.
Simultaneously, plasma transfusions were done when INR level
was greater than 2.5. Thrombophilia testing demonstrated normal
activities of antithrombin and protein C, S and she did not have FV
Conclusions: FV defects can cause bleeding and thrombosis at the
same time and FV deficiency presents with variable clinical symptoms.
Although prolonged coagulations tests were detected, thrombosis is a
challenge in patients with FV deficiency