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CASE REPORT
Year : 2018  |  Volume : 7  |  Issue : 2  |  Page : 140-142

Elevated factor VIII and mesenteric vein thrombosis


Department of Gastroenterology, KGH, Andhra Medical College, Visakhapatnam, Andhra Pradesh, India

Date of Web Publication6-Jun-2018

Correspondence Address:
Dr. K Sravan Kumar
Department of Gastroenterology, KGH, Andhra Medical College, Visakhapatnam, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JDRNTRUHS.JDRNTRUHS_75_16

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  Abstract 


Elevated concentrations of factor VIII have recently been associated with de novo development of deep vein thrombosisand its recurrence Occurrence of mesenteric vein thrombosis in patients with elevated factor VIII has been rarely described but not well characterized. We describe in this article a young patient who developed superior mesenteric vein thrombosis and on evaluation was found to have abnormally elevated factor VIII.

Keywords: Anticoagulation,factor VIII, mesenteric vein thrombosis


How to cite this article:
Krishna P M, Kumar K S, Girinidh L R, Kumar AR. Elevated factor VIII and mesenteric vein thrombosis. J NTR Univ Health Sci 2018;7:140-2

How to cite this URL:
Krishna P M, Kumar K S, Girinidh L R, Kumar AR. Elevated factor VIII and mesenteric vein thrombosis. J NTR Univ Health Sci [serial online] 2018 [cited 2019 Dec 11];7:140-2. Available from: http://www.jdrntruhs.org/text.asp?2018/7/2/140/233849




  Introduction Top


EHPVO is a vascular disorder of the liver. It is defined by obstruction of the extra-hepatic portal vein with or without involvement of the intra-hepatic portal veins or splenic or superior mesenteric veins.[1] Elevated plasma levels of coagulation factor VIII, after prothrombin and factor V, is the next most common cause of thrombophilia. It is present in 20% of patients with deep vein thrombosis(DVT)of the lower limbs increasing the risk of the disease in a dose dependent manner.[2] Like fibrinogen, factor VIII is an acute phase reactant whose concentration rises in plasma in the presence of inflammation. However, high factor VIII levels are associated with an increased risk of DVT independent of the acute phase reaction.[3]


  Case Report Top


A 21-year-old male in good health and no significant past medical history presented with abdominal pain for 2 months in epigastric and left hypochondrium. He was not on any drugs. There was a history of similar complaints in his younger sister who died 2 years back without any evaluation. A contrast-enhanced computed tomography scan of the abdomen showed superior mesenteric vein thrombosis with multiple collaterals in the perigastric and porta hepatitis [Figure 1].
Figure 1: Contrast enhanced computed tomography scan of the abdomen showing superior mesenteric vein thrombosis with multiple collaterals in perigastric and porta hepatis

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A coagulation profile work-up was done. His factor VIII activity concentration was found to be 260% of the normal (normal: 60–150). Analyses for prothrombin O20210A mutation, factor V Leiden mutation, and activated protein C resistance were all negative. Protein C and S activities and homocysteine concentrations were normal. Anti-cardiolipin antibody and screen for lupus anticoagulant were negative. Thrombin time was normal, as were fibrinogen, plasminogen activator inhibitor-1 activity, and tissue plasminogen activator antigen. Flow cytometry for paroxysmal nocturnal hemoglobinuria was negative. An acute phase reaction as a cause of increased factor VIII levels was excluded in the patient because of constant normal body temperature, white blood cell count, and C-reactive protein levels.

Upper endoscopy showed no esophageal and gastric varices. The patient was started on warfarin for anticoagulation as his abdominal pain was very severe. After starting anticoagulant drugs, his abdominal pain decreased; at present, he is asymptomatic.


  Discussion Top


Specific coagulation defects are increasingly recognized as the precipitating factors for cases of mesenteric vein thrombosis previously thought to be idiopathic.[4] Among these are factor V Leiden gene mutation, prothrombin G20210A gene mutation, antiphospholipid syndrome, hyperhomocysteinemia, and deficit of protein C and S.[5] Some patients may have multiple defects as coexpression of different thrombophilic genes.[6]

Recently, increased levels of factor VIII have been identified as an independent risk factor of recurrent venous thromboembolism (thromboses in axillary veins, distal and proximal veins of the leg, pulmonary embolism). It has been estimated that approximately 16% cases of DVT in the general population are the result of elevated factor VIII concentration, with a prevalence of 25% during the first episode.[7]

To our knowledge, there are three reported cases reported of portal vein thrombosis associated with elevated factor VIII. One patient was a 33-year-old man from Germany who suffered partial thrombosis of the portal vein and superior mesenteric vein and was found to have elevation of both factor VIII and von Willebrand factor.[8] The second was a female patient, also from Germany, with a history of splenic vein thrombosis at age 18 treated with splenectomy, followed by recurrent portal and superior mesenteric vein thrombosis at age 31. Interestingly this patient was on birth control therapy at the time of the splenic vein thrombosis. Another case was of a 41-year-old female on oral contraceptives from the US with portal, splenic, and superior mesenteric vein thrombosis.[9]

In the Leiden Thrombophilia Study, women with high factor VIII: C concentration and concomitant oral contraceptive use had a 10.3 fold increased risk of venous thrombosis [Odds ratio (OR) 10.3; 95% confidence interval (Cl) 3.7–28.9) compared with a 4-fold increased risk (OR 4.0; 95% Cl 2.0–8.0) in women with high factor VIII: C concentration but no oral contraceptive use.[10] This may reflect the “multihit” effect of individual hypercoagulable factors on the overall risk of venous thrombosis. Moreover, both factors may act independent of each other to increase the risk of DVT because hormone replacement therapy was not found to be associated with an increase in factor VIII concentration in a recent randomized placebo-controlled trial.[11]

Long-term management of patients with elevated factor VIII who suffered a prior episode of DVT remains problematic. There is evidence that patients with factor VIII concentrations above the 90th percentile have a 37% recurrence rate of thrombosis at 2 years. Considering this high recurrence rate at factor VIII concentrations above the 90th percentile and the severity of this patient's initial thrombotic event, we would recommend continuing anticoagulation indefinitely in such patients. The method of anticoagulation should be selected on an individual basis according to the presence of clinical conditions predisposed to bleeding such as esophageal and gastric varices; to reduce the bleeding risk for patients with varices, it would be prudent to use medication that has a more predictable anticoagulant activity and shorter halflife, i.e., unfractionated or low-molecular-weight heparin rather than warfarin. Early initiation of anticoagulation is also recommended to increase the chance of recanalization.

To our knowledge, this is the first report on the association of increased factor VIII levels and isolated superior mesenteric vein thrombosis.

We conclude that increased levels of factor VIII may play a pathogenic role in the development of portal, splenic, and mesenteric thrombosis. Therefore, factor VIII should be introduced in the diagnostic work-up of idiopathic splanchnic thrombotic events.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Sobhonslidsuk A, Reddy KR. Portal vein thrombosis: A concise review. Am J Gastroenterol 2002;97:535-41.  Back to cited text no. 1
[PUBMED]    
2.
Koster T, Blann AD, Briet E, Vandenbroucke JR Rosendaal FR. Role of clotting factor VIII in effect of von Willebrand factor on occurrence of deep-vein thrombosis. Lancet 1995;345:152-5.  Back to cited text no. 2
    
3.
Kraaijenhagen RA, in't Anker PS, Koopman MMW, Reitsma PH, Prins MH, van den Ende A, et al. High plasma concentration of factor VIIIc is a major risk factor for venous thromboembolism. Thromb Haemost 2000;83:5-9.  Back to cited text no. 3
    
4.
Denninger MH, Chait Y, Casadevall N, Hillaire S, Guillin MC, Bezeaud A, et al. Cause of portal or hepatic venous thrombosis in adults: The role of multiple concurrent factors. Hepatology 2000;31:587-91.  Back to cited text no. 4
    
5.
Blendis L, Wong F. Hepatoportal thromboses are no longer idiopathic. Gastroenterology 2000;119:874-5.  Back to cited text no. 5
[PUBMED]    
6.
Madonna P, De Stefano V, Coppola A, Cerbone AM. G2021OA prothrombin gene mutation and other trombophilic polymorphisms in patients with portal or hepatic venous thrombosis. Gastroenterology 2001;120:1059-67.  Back to cited text no. 6
    
7.
Kamphuisen PW, Eikenboom JCJ, Bertina RM. Elevated factor VIII levels and the risk of thrombosis. Arterioscler Thromb Vase Biol 2001;21:731-8.  Back to cited text no. 7
    
8.
Gunther R, Folsch UR. High plasma levels of factor VIII and von Willebrand Factor in a patient with portal vein thrombosis. Z Gastroenterol 2002;40:409-12.  Back to cited text no. 8
    
9.
Julapalli VR, Bray PF, Duchini A. Elevated Factor VIII and Portal Vein Thrombosis. Dig Dis Sci 2003;48:2369-71.  Back to cited text no. 9
[PUBMED]    
10.
Bloemenkamp KVM, Heimerhorst FM, Rosendaal FR, Vandenbroucke JP. Venous thrombosis, oral contraceptives and high factor VIII levels. Thromb Haemost 1999;82:1024-7.  Back to cited text no. 10
    
11.
Hoibraaten E, Qvigstad E, Andersen TO, Mowinckel MC, Sandset PM. The effect of hormone replacement therapy (HRT) on hemostatic variables in women with previous venous thromboembolism results from a randomized, double-blind, clinical trial. Thromb Haemost 2001;85:775-81.  Back to cited text no. 11
    


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