Haemostatic parameters in patients with behget's disease

This study aimed to evaluate the cause of thrombosis in Behcet's disease [BD] patients, since abnormalities in coagulation and fibrinolytic parameters have shown contradictory results. Haemostatic parameters were retrospectively evaluated in BD patients treated between January 2007 and January 2011 at Sultan Qaboos University Hospital, Oman. The blood samples of 35 Omani BD patients and 30 healthy controls were analysed for factor VIII:C levels, activated protein C resistance [APCR], von Willebrand factor [vWF] antigens [Ag], collagen binding and ristocetin co-factor activity [RiCoF], antithrombin [AT] protein C [chromogenic and clotting], protein S, homocysteine, tissue plasminogen activator, plasminogen activator inhibitor, plasminogen, alpha 2-antiplasmin, lupus anticoagulant and anticardiolipin and beta2-glycoprotein-l antibodies. The mean values of factor VIII:C, vWF Ag, AT and protein S were significantly higher in the patient group [P = 0.01, 0.006, 0.04 and 0.01, respectively]. There was no deficiency in protein C. Screening for APCR, anticardiolipin antibodies, anti-beta2-glycoprotein-l antibodies and lupus anticoagulant was negative and there were no differences in homocysteine levels, nor were there differences between patients with and without thrombosis. Six patients had elevated factor VIII:C levels [>150 lU/dL, P <0.02] which normalised on repeat measurements after three months. The elevation of factors VIII:C, vWF Ag and AT most likely represent an acute phase phenomenon. In this study, thrombophilic factors did not seem to explain thrombotic tendency. Therefore, further mechanistic studies in a larger group of patients are needed to elucidate the basis for thrombosis in BD. We hypothesise that active BD causes vasculitic endothelial perturbation with dysfunction, leading to the observed increased propensity for thrombosis

Diagnosis of beta-thalassaemia carriers in the Sultanate of Oman

Haemoglobinopathies are a major cause of morbidity in the Sultanate of Oman and premarital screening is being encouragedin order to reduce the number of affected births. The identification of beta-thalassaemia carrier status is an essential prerequisite of any screening programme. However, the level of Haemoglobin [Hb] A[2], which is used to detect beta-thalassaemia carriers, can be affected by other factors including iron deficiency, concurrent alpha thalassaemia and the type of DNA mutation present. The following study was undertaken to ascertain if the Hb A[2] level is an appropriate tool for the identification of beta-thalassaemia carriers in the Omani population. Hb A[2] was measured by high performance liquid chromatography [HPLC] in 160 obligate carriers of beta-thalassaemia. 158 subjects had Hb A[2] levels above 3.5% indicating beta-thalassaemia trait. Two subjects had slightly lower levels and were found to be iron deficient. After therapy both these subjects' Hb A[2] levels increased to above 3.5%. In the absence of iron deficiency, Hb A[2] is an accurate marker for the presence of beta-thalassaemia trait in the Sultanate of Oman