Published: 2022-09-23

Challenges in diagnosis and management of acquired factor V inhibitors

Ruchi Gupta, Dinesh Chandra, Sanjeevan Sharma, Priyanka Mishra


Inhibitors to factor V is a rare phenomenon with varied clinical presentation ranging from asymptomatic states to life-threatening bleeds. They are known to be associated with exposure to bovine thrombin, drugs, autoimmune diseases and malignancies. Establishing the diagnosis of FV inhibitors is challenging and the presence of lupus-like properties of the inhibitor can further complicate the diagnosis. Here we document an unusual case of an asymptomatic elderly female posted for pacemaker implantation and incidentally, the laboratory workup revealed a disproportionately abnormal coagulation screen. The intricacies in the diagnosis and management are discussed along with a brief review of the literature. An awareness of the diverse manifestations of this underrecognized disorder and difficulties in management is essential for medical practitioners, particularly in patients with idiopathic severe bleeding diathesis.


Factor V inhibitor, Lupus-like inhibitor, Acquired, Idiopathic, Anti-coagulant effect

Full Text:



Ang AL, Kuperan P, Ng CH, Ng HJ. Acquired factor V inhibitor. A problem-based systematic review. Thromb Haemost. 2009;101(5):852-9.

Franchini M, Lippi G. Acquired factor V inhibitors: a systematic review. J Thromb Thrombolysis. 2011;31(4):449-57.

Wiwanitkit V. Spectrum of bleeding in acquired factor V inhibitor: a summary of 33 cases. Clin Appl Thromb Hemost. 2006;12(4):485-8.

Kamal AH, Tefferi A, Pruthi RK. How to interpret and pursue an abnormal prothrombin time, activated partial thromboplastin time, and bleeding time in adults. Mayo Clin Proc. 2007;82(7):864-73.

Wang L, Nguyen A. Acquired factor V inhibitor with lupus-like features. Lab Med. 2002;33(9):551-2.

Kapur A, Kelsey PR, Isaacs PE. Factor V inhibitor in thrombosis. Am J Hematol. 1993;42(4):384-8.

Perdekamp MT, Rubenstein DA, Jesty J, Hultin MB. Platelet factor V supports hemostasis in a patient with an acquired factor V inhibitor, as shown by prothrombinase and tenase assays. Blood Coagul Fibrinolysis. 2006;17(7):593-7.

Ashizawa M, Kimura S, Wada H, Sakamoto K, Sato M, Terasako K, et al. Acquired factor V inhibitor associated with life-threatening bleeding and a mixing test result that indicated coagulation factor deficiency. Hematology. 2013;18(5):300-4.

Gould WR, Simioni P, Silveira JR, Tormene D, Kalafatis M, Tracy PB. Megakaryocytes endocytose and subsequently modify human factor V in vivo to form the entire pool of a unique platelet-derived cofactor. J Thromb Haemost. 2005;3(3):450-6.

Lebrun A, Leroy-Matheron C, Arlet JB, Bartolucci P, Michel M. Successful treatment with rituximab in a patient with an acquired factor V inhibitor. Am J Hematol. 2008;83(2):163-4.

Takemoto K, Hamada O, Kitamura K, Fujiwara N, Miyakawa Y. Challenges in management of unusual acquired factor V deficiency: A case report. Medicine (Baltimore). 2019;98(17):e15259.