Keklik Karadağ F. , Demirci Z., Arslan A. , Soyer N. , Saydam G. , Şahin F.

11th BIC International Conference (Advances in Haemostasis and Bleeding Disorders) Venice, Italy, September 17-19 2021, Venice, Italy, 17 - 19 September 2021, pp.27-28

  • Publication Type: Conference Paper / Summary Text
  • Doi Number: 10.3324/haematol.2021.s1
  • City: Venice
  • Country: Italy
  • Page Numbers: pp.27-28


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 leiden mutation. 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