ABSTRACT
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
ABSTRACT
The aim of this study was to validate the interpretation of red blood cell indices in complete blood count [CBC] and high performance liquid chromatography [HPLC] results on cord blood samples in consecutive Omani neonates. Cord blood samples from 7,837 neonates, were analysed with CBC and HPLC using the beta-thalassaemia short programme. Direct sequencing of abnormal samples with HbS, HbD, HbE and HbC was performed to validate the HPLC results. Additionally, in cases with HbA <10%, the beta-globin gene was directly sequenced for beta-thalassaemia mutation analysis. Overall, 4,042 subjects [51.58%] had normal HPLC [HbA 22.88 +/- 8.03; HbF 77.02 +/- 8.04], whereas the presence of Hb Barts in the remaining 3,795 cases [48.42%] indicated the presence of alpha-thalassaemia. No case of HbH was detected. In the former subgroup respectively, the mean Hb [15.38 +/- 2.04 g/dl] red blood cell [RBC] count [4.69 +/- 0.68 x 10[12]/l], Hct [50.5 +/- 7.18%], mean corpuscular volume [MCV] [107.66 +/- 7.75 fl], mean corpuscular haemoglobin [MCH] [33.31 +/- 4.07 pg], mean corpuscular haemoglobin concentration [MCHC] [30.98 +/- 3.44 g/dl], red cell distribution width [RDW] [17.01 +/- 2.17%] whereas, in the latter group with alpha -thalassaemia, it was [14.79 +/- 2.90 g/dl]; [5.09 +/- 0.77 x 10[12]/l]; [49.7 +/- 7.40%]; [97.29 +/- 13.8 fl]; [29.74 +/- 11.80 pg]; [30.39 +/- 3.6 g/dl], and [18.09 +/- 2.56%] respectively. DNA sequencing of samples with abnormal haemoglobin could validate the CBC and HLPC interpretations in all cases. This is the first study comparing the hemoglobin and red cell indices in the cord blood from newborn Omani subjects with those from other countries in the region, showing comparable results to those seen in Saudi neonates. The study also validates the CBC and HPLC interpretations of the cord blood red cell indices in the Omani neonate. The incidence of alpha-thalassaemia diagnosed by the presence of Hb Barts in cord blood of neonates was 48.42%