Thrombophilia and retinal vascular occlusion

Abstract

PurposeThe purpose of this research was to assess associations of thrombophilia with central retinal vein occlusion (CRVO), central retinal artery occlusion (CRAO), and amaurosis fugax (AF); to evaluate outcomes of normalizing high homocysteine; and to study CRVO, CRAO, and AF developing in estrogens/estrogen agonists in women subsequently shown to have thrombophilia.MethodsMeasures of thrombophilia–hypofibrinolysis were obtained in 132 CRVO cases, 15 CRAO cases, and 17 AF cases. Cases were compared to 105 healthy control subjects who did not differ by race or sex and were free of any ophthalmologic disorders. All cardiovascular disease (CVD) risk factors were compared to healthy general populations.Main outcome measuresThe main outcome measure of this study was thrombophilia.ResultsCRVO cases were more likely than controls to have high homocysteine (odds ratio [OR] 8.64, 95% confidence intervals [CI]: 1.96–38), high anticardiolipin immunoglobulin M (IgM; OR 6.26, 95% CI: 1.4–28.2), and high Factor VIII (OR 2.47, 95% CI: 1.31–7.9). CRAO-AF cases were more likely than controls to have high homocysteine (OR 14, 95% CI: 2.7–71.6) or the lupus anticoagulant (OR 4.1, 95% CI: 1.3–13.2). In four of 77 women with CRVO (two found to have high homocysteine, two with inherited high Factor XI), CRVO occurred after starting estrogen–progestins, estrogen–testosterone, or estrogen agonists. In one of eight women with CRAO found to have high anticardiolipin antibody IgG, CRAO occurred after starting conjugated estrogens, and AF occurred after starting conjugated estrogens in one of eleven women with AF (inherited protein S deficiency). Therapy for medians of 21 months (CRVO) and 6 months (CRAO-AF) was 5 mg folic acid, 100 mg B6, and 2000 mcg/day B12 normalized homocysteine in 13 of 16 (81%) CRVO cases and all five CRAO-AF cases with pretreatment hyperhomocysteinemia. The CRVO cases had an excess of hypertension; CRAO-AF cases had an excess of type 2 diabetes and hypertension.ConclusionTreatable thrombophilia, hyperhomocysteinemia in particular, is more common in RVO cases than in normal controls. RVO occurs after estrogens or estrogen agonists were administered in women subsequently shown to have thrombophilia.

Seasonality and Incidence of Central Retinal Vein Occlusion in Sweden: A 6-Year Study

“…Increased risk of CRVO has been associated with deficiency of protein C (Tekeli 1999), protein S (Yap 2007) and antithrombin III (Ririe 1979). Furthermore other studies have reported that factor V Leiden mutation (Rehak 2008) , antiphospholipid (Agarwal 2009) and anticardiolipin syndrome (Glueck 2012) hyperhomocysteinaemia (Cahill 2003) and elevated levels of plasminogen activator inhibitor-1 (Marcucci 2001) may play a role in the etiology of CRVO.…”

Systematic Treatment of Central Retinal Vein Occlusion

“…According to this hypothesis, smoking cessation is recommended. Hyperhomocysteinemia, a thrombophilic state identified and treated in our patient, is an independent risk factor of CRVO [2]. The treatment consists of vitamin supplementation, folic acid 5 mg/day.…”

“…Complete blood count was normal. Further blood tests revealed hyperhomocysteinemia [2], which has been identified as independent risk fac-tors for the occurrence of recurrent CRVO. The patient was referred to a specialist for a carotid Doppler ultrasound and echocardiography in order to exclude embolic sources.…”

Bilateral BRVO in a patient with recurrent prostate cancer

“…In 2005, a comprehensive meta-analysis found a clear association only with antiphospholipid syndrome and a lesser relationship with factor V Leiden and mutation of the prothrombin gene 13. More recently, a retrospective study of 164 cases found higher levels of homocysteinaemia, anticardiolipin antibodies and inherited factor VIII in addition to lower inherited protein S in RVO compared to controls 14. In a similar mechanism, infectious and inflammatory diseases including hepatitis or cardiac graft rejection have been associated with bilateral vein occlusions 5…”