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Thrombosis and the hypercoagulable state.

Our understanding of thrombosis remains anchored to Virchow's view that the evolution of a thrombus is fostered by vascular damage, by retarded blood flow, and by alterations in the blood itself that increases its likelihood to clot. The last of these forces, the so-called hypercoagulable state, has been defined in many ways. For example, some evidence exists that increased titers of clotting factors or increased numbers of circulating platelets may be correlated with a thrombotic tendency. A more exciting way that clotting factors might contribute to thrombosis is that under certain conditions the titer of their activated forms might reach critical levels in the circulation. Another possibility is that a thrombotic tendency may be caused by qualitative alterations in clotting factors or platelets. The firmest evidence that alterations in blood clotting may be related to thrombosis is the undoubted high incidence of recurrent thromboembolism in patients with familial deficiencies of antithrombin III or protein C, an inhibitor of antihemophilic factor (factor VIII) and proaccelerin (factor V). Impaired fibrinolytic mechanisms may also predispose to thrombosis. Not yet explained is the fact that patients with lifelong hemostatic alterations in peripheral blood sustain only a handful of thrombotic episodes.

Blood Coagulation↗

Hypercoagulable state under low-intensity warfarin anticoagulation assessed with hemostatic markers in cardiac disorders.

The hemostatic condition under low-intensity anticoagulation in cardiac disorders is not fully elucidated. The aim of this study was to ascertain whether hemostatic molecular markers are a useful assessment for anticoagulation to detect the hypercoagulable state. A hematologic study was performed in 75 outpatients, without thromboembolic episodes, treated with low-intensity anticoagulation (average international normalized ratio [INR] 1.72) because of potential cardiac sources of arterial emboli, and in 40 age-matched control subjects. The average level of thrombin-antithrombin III complex (TAT) was significantly lower in patients than in control subjects (p = 0.005), and the mean value of D-dimer was not statistically different between patients and control subjects. Although TAT correlated moderately with D-dimer (r = 0.45, p = 0.0001), INR did not correlate with TAT or D-dimer. Elevated TAT > 3.0 ng/ml and/or D-dimer S 150 ng/ml were observed in 15 patients (20.0%), whereas the remaining 60 patients (80.0%) had no obvious increase in the level of TAT or D-dimer at overall INR. Antithrombin III activity did not correlate significantly with INR, but protein C activity and free protein S antigen showed a significant negative relation to INR (r = 0.82, r = 0.62, respectively, p = 0.0001). Low-intensity anticoagulation was sufficient to reduce coagulation and subsequent fibrinolytic activation in cardiac disorders, but may not be sufficient in some patients with elevated TAT or D-dimer concentration.(ABSTRACT TRUNCATED AT 250 WORDS)

Antithrombin III↗

Antithrombin III infusion suppresses the hypercoagulable state in adult acute lymphoblastic leukaemia patients treated with a low dose of Escherichia coli L-asparaginase. A GIMEMA study.

Thrombotic events have been reported in acute lymphoblastic leukaemia patients, especially during or after L-asparaginase administration. A so-called L-asparaginase associated coagulopathy has been well recognized, being characterized by a hypercoagulable state (decrease of antithrombin III, plasminogen, protein C, protein S and increase of prothrombin fragment F1 + 2, thrombin-antithrombin complexes and fibrinopeptide A). The aim of this study was to determine whether the supplementation of antithrombin III (AT-III) concentrates could improve the L-asparaginase associated coagulopathy, thereby blocking the activation of the haemostatic system. In 25 adult patients with acute lymphoblastic leukaemia (M 19, F6, mean age 34 years) antithrombin III (AT-III) concentrates were administered at daily doses of 50 U/kg for 10 consecutive days from the beginning of L-asparaginase therapy (6,000 U/m2/day s.c. for 7 days), given according to the GIMEMA ALL 0288 trial. A marked increase of antithrombin III was recorded on days IV-VIII-XI (P < 0.001). No changes in protein C, protein S, plasminogen, alpha 2-antiplasmin, factor VII and platelet count were observed and there was no increase in markers of hypercoagulability. There was no evidence of disseminated intravascular coagulation. In conclusion, AT-III concentrate supplementation during L-asparaginase therapy, by the achievement of high levels of antithrombin III, is associated with a lack of activation of the haemostatic system and appears to overcome the complex coagulopathy associated with L-asparaginase.

Adolescent↗

Diabetes mellitus as a hypercoagulable state: its relationship with fibrin fragments and vascular damage.

Haemostatic variables were assessed in 43 patients, 28 insulin-dependent and 15 non insulin-dependent. Maximum aggregation by low concentrations of adenosine diphosphate (ADP) or arachidonic acid and elevated plasma concentrations of TxB2, Factor VIII, vWF:Ag, RCoF and fibronectin (Fnct) indicated a hypercoagulable state. The manifestation of vasculopathy was associated with elevated concentrations of RCoF, Fnct, Hbalc, cholesterol and triglycerides, while impaired fibrinolysis was demonstrated by decreased t-PA levels and the absence of crosslinked fibrin degradation products (XL-FDP).

Adolescent↗

Reversal of low-molecular-weight heparin-induced bleeding in patients with pre-existing hypercoagulable states with human recombinant activated factor VII concentrate.

Low-molecular-weight heparins are widely employed in prophylactic and therapeutic antithrombotic regimens for venous thromboembolic events. Excessive anticoagulation with low-molecular-weight heparins rarely can precipitate catastrophic bleeding complications. Currently, there is no specific or reliable antidote that can reverse the anticoagulant effects of low-molecular-weight heparins efficiently and safely. This report describes three individuals with underlying hypercoagulable states, who developed clinically significant bleeding complications while receiving therapeutic anticoagulation with enoxaparin. All of the hemorrhagic events subsequently were safely and effectively reversed with a single intravenous bolus infusion of recombinant activated factor VIIa (RFVIIa) concentrate. Hemoglobins, prothrombin times, and clinical overt bleeding were monitored before and after the administration of RFVIIa. In all three cases, bleeding was controlled without an increase in thrombotic events. Our findings demonstrate that RFVIIa can rapidly and safely reverse the hemorrhagic adverse effects associated with excessive levels of low-molecular-weight heparin in patients with pre-existing hypercoagulable conditions and/or acute venous thromboembolism.

Adult↗

Deep vein thrombosis: the risks of sclerotherapy in hypercoagulable states.

There have been many case reports of deep vein thrombosis and pulmonary thromboembolism in patients undergoing sclerotherapy for the treatment of varicose veins, but the true incidence of venous thromboembolism in this patient population is unknown. Patients who develop deep vein thrombosis while being treated for varicose veins do so either because of an error in treatment or because the patient suffers from some underlying hypercoagulable state. It is possible to identify prospectively many patients who are at especially high risk for venous thromboembolism by virtue of genetic predisposition, past or recent medical problems, or environmental factors. When sclerotherapy is used in such patients, special efforts must be made to protect against the development of venous thrombosis during treatment.

Blood Coagulation Disorders↗

Comprehensive hypercoagulable state testing is indicated in patients with a first idiopathic deep venous thrombosis.

A 45-year-old man presents with a 3-day history of right leg swelling and pain. He is diagnosed with an acute right common femoral vein thrombosis. He takes no medications, has an unremarkable medical history, no recent trauma or surgery, no recent travel, and no known cancer. Performance of a comprehensive hypercoagulable state panel is contemplated.

Blood Coagulation Disorders↗

Thrombosis and a hypercoagulable state in HIV-infected patients.

Human immunodeficiency virus infection is an illness with protean manifestations including hematological abnormalities. Thromboembolic complications in HIV-infected patients have been described. Recent literature describes an incidence ranging from 0.26% to 7.6%; higher incidence is seen in patients with active opportunistic infections or malignancy, and in patients with the acquired immunodeficiency syndrome. A variety of potential mechanisms have been proposed to account for the observed hypercoagulability in HIV-infected patients. These include the presence of antiphospholipid-anticardiolipin antibodies, decreased activities of natural anticoagulants (especially protein S), and increased platelet activation. Recent epidemiological studies emphasize the increased incidence of thromboembolic events including myocardial infarction in the HIV-infected population after the introduction of highly active antiretroviral therapy. The use of protease inhibitors in particular is implicated. A hypercoagulable state and especially thromboses are emerging as clinical issues in HIV-infected patients. Further studies are in order to more clearly delineate the pathophysiologic mechanism(s) of thromboses in HIV-infected patients.

Adult↗

The role of hyperglycaemia and the hypercoagulable state in the pathogenesis of cardiovascular events in diabetes mellitus: implications for hypertension management.

Population studies have identified diabetes mellitus as a risk factor for cardiac and vascular events, with a common association with hypertension. Observational studies have consistently demonstrated blood glucose as a continuous cardiovascular risk factor. Experimental studies suggest that elevated blood glucose, through a range of biochemical pathways can promote atherogenesis and increase the tendency for thrombosis. The resultant state promotes occlusion of arterial flow and increase cardiovascular event rates. However, an unconvincing temporal relationship (raised blood glucose preceding cardiovascular events) and the limited impact of glucose reduction in patients with diabetes on cardiovascular events do not support blood glucose as a major cause of cardiovascular disease. In this review, we discuss the relationship between hyperglycaemia and a hypercoagulable state in diabetes and the implications of this relationship for the treatment of patients with diabetes, who also commonly have hypertension.

Blood Coagulation↗

Further studies on the hypercoagulable state of patients with Cushing's syndrome.

Factors XII, XI, IX and VIII, plasminogen and alpha 2-antiplasmin levels were found to be increased in a group of patients affected by Cushing's syndrome. High activity of these coagulation factors could be due to their increased release and synthesis mediated by cortisol. A significant correlation between the main arterial pressure and either factor VIII antigen, ristocetin cofactor or factor XII activity was found. Moreover a similar correlation between factor XII activity and either factor VIII antigen or ristocetin cofactor was seen. In conclusion, the presence of a hypercoagulable state in Cushing's syndrome seems confirmed. Synergic release of factor XII and factor VIII from endothelium could be due to high blood vessel tone secondary to hypercorticism. Finally, decreased fibrinolytic activity was suspected according to plasminogen and alpha 2-antiplasmin increase.

Blood Coagulation Disorders↗

Effect of all-trans-retinoic acid on the hypercoagulable state of patients with breast cancer.

To evaluate whether all-trans-retinoic acid (ATRA) is able to modulate the hemostatic system in patients with solid tumors, we studied patients with locally advanced breast cancer who were enrolled in a Phase Ib study of ATRA +/- Tamoxifen (Tam). In this study, two groups of 15 patients/each were treated for 21 days before operation with ATRA at three doses (15, 45, or 75 mg/m(2)/day on alternate days) given alone (group 1) or in combination with Tam (group 2). One additional group received Tam alone. Plasma samples were evaluated for hypercoagulation markers (FVIIa, F1+2, TAT, D-dimer), fibrinolysis proteins (t-PA, PAI-1), and coagulation inhibitors (protein C, AT). At baseline, cancer patients had FVIIa, F1+2, TAT, and PAI-1 significantly greater than control subjects. During treatment, in the patients given ATRA alone, hypercoagulation markers appeared unmodified. Instead, subjects given Tam alone had a significant elevation of FVIIa, F1+2, and TAT versus baseline. However, in the ATRA + Tam groups, hypercoagulation markers were decreased compared with Tam alone. These results suggest that in selected conditions, pre-operative ATRA may modulate the hypercoagulable state of breast cancer patients.

Aged↗

Does heart failure confer a hypercoagulable state? Virchow's triad revisited.

It is well-recognized that patients with congestive heart failure are at an increased risk of stroke and venous thromboembolism. Nevertheless, stroke, thromboembolism and myocardial infarction have generally been regarded to be end points of secondary importance in large heart failure trials, when compared with mortality or hospital readmissions. It may well have been that the incidence of thrombotic events are underestimated. The problem of thrombus formation (thrombogenesis) in heart failure may therefore be a much more significant problem than is currently recognized. The pathophysiology of thrombogenesis in heart failure could well be explained in the context of Virchow's original triad. In addition to "abnormal flow" through low cardiac output, dilated cardiac chambers and poor contractility, patients with heart failure also demonstrate abnormalities of hemostasis and platelets (that is "abnormal blood constituents") and endothelial dysfunction ("vessel wall abnormalities"). These abnormalities contribute to a prothrombotic or hypercoagulable state, which increases the risk of thrombosis in heart failure and impaired left ventricular systolic function. Some observational data are available on the role of anticoagulants in heart failure, and there is sound evidence to support the use of antithrombotic therapy in patients with heart failure and atrial fibrillation. However, there are no large-scale prospective randomized controlled trials of antithrombotic therapy in patients with heart failure who remain in sinus rhythm, although important studies are in progress. Although the results of these studies are awaited, measurement of suitable markers of thrombogenesis might prove to be valuable in identifying "high risk" patients and in determining the nature, duration and intensity of such treatment. Further information is also needed on the predictive value of various markers of hypercoagulability in patients with heart failure, the association between hemostatic variables and the severity of heart failure, and the effects of different treatments.

Anticoagulants↗

Hypercoagulable state and thromboembolism following warfarin withdrawal in post-myocardial-infarction patients.

Nine out of 47 (19%) patients on chronic anticoagulation with warfarin, as secondary prophylaxis after myocardial infarction, initially treated with streptokinase, had thromboembolic complications within 4 weeks after sudden (7/25) or gradual (2/22:NS) warfarin withdrawal. The biochemical effects of warfarin withdrawal were repeatedly studied in 20 of the patients during the first 14 days following drug cessation. During the first 4 days, the levels of coagulation factors VII and IX increased more rapidly than proteins C and S. Thus, a gap was created between the factors provoking and inhibiting the coagulation process. Furthermore, plasma concentrations of fibrinopeptide A (FPA) increased, reflecting activation of the coagulation system. These laboratory findings suggest that withdrawal of warfarin creates a transient hypercoagulable state, imposing a risk of thromboembolic events in patients given anticoagulant treatment as secondary prophylaxis following myocardial infarction.

Adult↗

Hypercoagulable state and disseminated intravascular coagulation following an effective chemotherapy in tumor-bearing rats.

OBJECTIVE: To detect the changes in blood coagulation system and clarify the related mechanisms of chemotherapy-induced disseminated intravascular coagulation. METHODS: Changes in blood coagulation system and immunohistochemistry for fibrinogen were investigated in six groups of rats designed for different purposes. RESULTS: Decreased platelet count, prolonged prothrombin time and active partial thromboplastin time, elevated fibrinogen level, and decreased antithrombin were observed in the rats receiving a newly developed chemotherapy (NDC group), in which mitomycin C was administered intravenously together with angiotensin. Accumulation of fibrinogen and microthrombi in the blood vessels of multiple organs were also found in the NDC group by immunohistochemistry and histopathological examination. CONCLUSIONS: Rapid reduction of tumor mass induced by an effective chemotherapy could cause hypercoagulable state and disseminated intravascular coagulation.

Angiotensins↗

Diabetes, a hypercoagulable state? Hemostatic variables in newly diagnosed type 2 diabetic patients.

37 type 2 diabetic patients with no clinical evidence of retinopathy or vascular disease were studied at diagnosis and following control of hyperglycaemia for evidence of abnormalities of coagulation, fibrinolysis and platelet behaviour. 38% showed hyperactive platelets, demonstrating either in vitro hyperaggregability, circulating platelet aggregates, or raised plasma beta-thromboglobulin levels. 36% showed abnormally raised factor VIII coagulant activity (FVIIIc) levels, though this was mainly in female patients. The mean level of FVIIIc decreased with treatment. Anti-thrombin III (AT-III) levels were decreased, and 33% of the patients had levels less than 80%. In this group AT-III increased following treatment. No abnormalities of fibrinolysis were demonstrated. These findings support the concept that diabetes can be associated with a hypercoagulable state, which is not necessarily dependent on the presence of overt vascular disease, or correlated with the degree of chronic hyperglycaemia (HbA1c levels).

Adenosine Diphosphate↗

Hypertension associated with reduced plasma thrombomodulin levels and a hypercoagulable state in rats.

The plasma thrombomodulin (TM) level, an indicator of systemic endothelial cell damage, was measured in spontaneously hypertensive rats (SHR), deoxycorticosteron acetate (DOCA)-induced hypertensive rats and normotensive Wistar-Kyoto (WKY) rats to clarify its changes in hypertension. Plasma TM levels, measured by enzyme-linked immuno-sorbent assay, decreased with aging (5-20-weeks-old) in both SHR and WKY, and they were lower in SHR than age-matched WKY in all ages examined. Deoxycorticosteron acetate-induced hypertensive WKY also showed decreased TM levels compared with normotensive WKY. Accelerated coagulation and fibrinolysis shown by the increases in thrombin-antithrombin complex (TAT) and D-dimer levels were observed in both groups of hypertensive rats. These results suggest that hypertension may decrease plasma TM levels and induce a hypercoagulable state in rats.

Animals↗

Clinical significance of activated protein C resistance as a potential marker for hypercoagulable state.

The activated protein C (APC)-resistance test is a simple and reliable method for detecting reduced sensitivity to the anticoagulant action of this protein. We investigated the sensitivity to APC in 180 Japanese controls and in 96 Japanese patients with venous and arterial thrombosis (28 with deep vein thrombosis; 13 with pulmonary thromboembolism; 41 with cerebral infarction; and 14 with coronary artery disease). All of the patient groups showed significantly reduced sensitivity to APC, reflected by the lower normalized APC-sensitivity ratio (n-APC-SR), as compared with healthy control. The APC-sensitivity ratio was negatively correlated with plasma activated factor VII levels. These results suggest that the low n-APC-SR is related to venous or arterial thrombotic disease. The APC resistance may serve as a potential marker for assessing the hypercoagulable state.

Adult↗

Congenital antithrombin III deficiency: insights into the pathogenesis of the hypercoagulable state and its management using markers of hemostatic system activation.

Hereditary antithrombin III (ATIII) deficiency predisposes patients to venous thrombosis. The prothrombin fragment F1+2 radioimmunoassay demonstrates that many asymptomatic patients with this disorder not receiving antithrombotic therapy have elevated plasma factor Xa activity. The hemostatic system hyperactivity as measured by this assay could be specifically corrected by rising plasma ATIII levels of several persons into the normal range. This indicates that the prethrombotic state can be defined as an imbalance between the production and inhibition of factor Xa enzymatic activity. The effects of warfarin on factor Xa enzymatic activity in persons with congenital ATIII deficiency have also been evaluated. At equivalent intensities of oral anticoagulation, the mean plasma F1+2 level in patients with ATIII deficiency was significantly elevated as compared with anticoagulated persons without this inherited thrombotic disorder. It is concluded that the effect of warfarin on hemostatic system activation is modulated by the endogenous heparan sulfate-ATIII mechanism. This suggests that the F1+2 radioimmunoassay can be employed to improve the understanding of the hypercoagulable state associated with antithrombin III deficiency as well as to develop more effective treatment strategies to prevent thromboembolic events in patients with this disorder.

Antithrombin III Deficiency↗