Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.4.21.5 (thrombin)
 33,306 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A patient, aged 34, with a relapse of Behcet's disease (BD) showed laboratory features of enhanced coagulation activation without any symptoms of arterial or venous thrombosis. Elevated levels of thrombin-antithrombin III complexes (TAT), prothrombin fragments 1 + 2 (F1 + 2), associated with impairement of fibrinolysis were found. Total fibrinolytic activity, measured on fibrin plates, plasma concentration of tissue-type plasminogen activator (tPA:Ag) and plasmin-alpha 2-antiplasmin complexes (PAP) were low, whereas plasminogen activator inhibitor 1 concentration (PAI-1:Ag) was elevated in comparison with normal values. There intravenous 500 mg doses of cyclophosphamide, given on day 0, 7 and 50, resulted in a marked clinical improvement. This was accompanied by the normalization of augmented thrombin generation and increased fibrinolytic activity. This is the first report to show prothrombotic tendency in a relapse of Behcet disease and beneficial effect of cyclophosphamide therapy on haemostatic abnormalities. A degree of activation of coagulative and fibrinolytic system seems to be of importance in the pathogenesis of Behcet's disease, and monitoring of hemostatic parameters could be helpful in clinical assessment.
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PMID:[Subclinical pro-thrombotic state in a relapse of Behcet's disease--case report]. 912 17

Behcet's disease is a chronic inflammatory multi-systemic vasculitis. Recurrent oral and genital aphthous ulcers, uveitis, arthritis, arterial aneurysms, venous thrombosis, gastrointestinal system lesions and skin lesions can be seen. Large, medium or small arteries and veins may be involved. While venous lesions cause occlusion, arterial lesions can cause both occlusion and aneurysm. Major forms of pulmonary involvement include pulmonary artery aneurysm, arterial and venous thrombosis, pulmonary infarction, recurrent pneumonia, and pleurisy. In Behcet's disease, pulmonary thromboembolism (PE) is often associated with endothelial damage. Neutrophils play an important role in the pathogenesis of thrombosis. Coagulation components such as fibrinogen, thrombin, factor Xa and factor VIIa activate the inflammatory cascade and induce vascular events. It is important to understand the pathogenesis of vascular events in determining the effective treatment strategy. Here we present a patient with Behcet's disease who was presented with pulmonary embolism and was investigated for the etiology of thrombosis.
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PMID:Behcet's disease presenting with pulmonary thromboembolism. 3329 33