Korean J Hematol.  2008 Sep;43(3):190-193. 10.5045/kjh.2008.43.3.190.

A Case of Acquired Factor V Inhibitor

• Kim SY
• Kim HK
• Kim SW
• Cho HI

• KMID: 2252188
• DOI: http://doi.org/10.5045/kjh.2008.43.3.190

Abstract

Acquired inhibitor of coagulation factor V is a rare clinical condition and may cause significant morbidity and mortality. We report here the first Korean case of acquired isolated factor V inhibitor developed in a 73-year-old male patient after surgery for pancreatic head carcinoma. At post-operation day 2, intra-abdominal bleeding was recognized that was associated with a prolonged prothrombin time and activated partial thromboplastin time. The prolongation of both conditions was not corrected by a mixing test and factor V activity was reported to be 2%. Quantified titers of inhibitors were increased from 3.5 Bethesda unit to 8.4 Bethesda unit during follow-up analysis. In spite of the treatment with steroids and intravenous immunoglobulins, the case ended fatally. When acquired bleeding tendency with prolonged coagulation screening test is observed, evaluation of inhibitors of coagulation factors may be essential for the diagnosis and following appropriate management of the condition.

Keyword

Factor V; Blood coagulation factor; Inhibitor

MeSH Terms

Aged
Blood Coagulation Factors
Factor V
Follow-Up Studies
Head
Hemorrhage
Humans
Immunoglobulins, Intravenous
Male
Mass Screening
Partial Thromboplastin Time
Prothrombin Time
Steroids
Blood Coagulation Factors
Factor V
Immunoglobulins, Intravenous
Steroids

Reference

1). Ewenstein BM. Nonhemophilic inhibitors of coagulation. Kitchens CS, Alving BM, Kessler CM, editors. Consultative hemostasis and thrombosis. 2nd ed.Philadelphia, PA: Saunders Elsevier;2007. p. 89–90.

2). Knӧbl P., Lechner K. Acquired factor V inhibitors. Baillieres Clin Haematol. 1998. 11:305–18.

3). Streiff MB., Ness PM. Acquired FV inhibitors: a needless iatrogenic complication of bovine thrombin exposure. Transfusion. 2002. 42:18–26.

4). Taylor FB Jr., Toh CH., Hoots WK., Wada H., Levi M. Scientific Subcommittee on Disseminated Intravascular Coagulation (DIC) of the International Society on Thrombosis and Haemostasis (ISTH). Towards definition, clinical and laboratory criteria, and a scoring system for disseminated intravascular coagulation. Thromb Haemost. 2001. 86:1327–30.

5). Favaloro EJ., Posen J., Ramakrishna R, et al. Factor V inhibitors: rare or not so uncommon? A multi-laboratory investigation. Blood Coagul Fibrinolysis. 2004. 15:637–47.

6). Godart B., Boinot C., Remblier C., Hajjar A., Beau-chant M. Acquired factor V inhibitor associated with valproic acid use in a cirrhotic patients. Gut. 2006. 55:134–5.

7). Savage WJ., Kickler TS., Takemoto CM. Acquired coagulation factor inhibitors in children after topical bovine thrombin exposure. Pediatr Blood Cancer. 2007. 49:1025–9.

8). Ortel TL., Quinn-Allen MA., Charles LA., Devore-Carter D., Kane WH. Characterization of an acquired inhibitor to coagulation factor V. Antibody binding to the second C-type domain of factor V inhibits the binding of factor V to phosphatidylserine and neutralizes procoagulant activity. J Clin Invest. 1992. 90:2340–7.

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

10). Zumberg MS., Waples JM., Kao KJ., Lottenberg R. Management of a patient with a mechanical aortic valve and antibodies to both thrombin and factor V after repeat exposure to fibrin sealant. Am J Hema-tol. 2000. 64:59–63.

11). de Raucourt E., Barbier C., Sinda P., Dib M., Peltier JY., Ternisien C. High-dose intravenous immunoglobulin treatment in two patients with acquired factor V inhibitors. Am J Hematol. 2003. 74:187–90.

12). Bayani N., Rugina M., Haddad-Vergnes L., Lelong F. High-titer acquired factor V inhibitor responsive to corticosteroids and cyclophosphamide in a patient with two malignant tumors. Am J Hematol. 2002. 71:33–6.