Increased Level of Factor VIII and Physiological Inhibitors of Coagulation in Patients with Sickle Cell Disease

Chekkal, Mohamed; Rahal, Mohamed Chakib Arslane; Moulasserdoun, Khedidja; Seghier, F.

doi:10.1007/s12288-016-0701-z

Cited by 7 publications

(6 citation statements)

References 14 publications

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“…The mean comparison of FVIII inhibitor was significantly higher statistically (p<.01) in VOC condition than steady compared with the control group (Table 1). Our study agrees with previous reports [34][35], that factor VIII in SCA subjects havean isolated elevation, and it may explain the shortened activated partial thromboplastin time(aPTT) [36]. This finding is further supported by the fact that deficits at variable rates in physiological coagulation inhibitors and the increased activity of factor VIII are abnormalities of hemostasis that reflect the existence of a pro thrombotic state during vaso-occlusive state of SCA [26].…”

Section: Resultssupporting

confidence: 92%

Assessment of Some Haemostatic Parameters in Sickle Cell Anaemia Subjects Resident in Rivers and Bayelsa States

Mbeera

Akwuebu

Ezimah³

et al. 2021

JAMMR

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Aim: The aim of this study was to assess some haemostatic parameters in sickle cell anaemia subjects in Rivers and Bayelsa States. Study Design: This study is a cross-sectional observational study. Place and Duration of Study: This study was carried out at the University of Port Harcourt Teaching Hospital, Rivers State, and the Federal Medical Centre, Yenagoa, Bayelsa State, between the months of February and August, 2020. Methodology: A total of four hundred and fifty (450) subjects with age range of 1-50 years were randomly selected. There were about 200 registered patients (adults and children alike) at the sickle cell clinics of the University of Port Harcourt Teaching Hospital, and the Federal Medical Centre, Yenagoa, with an average of 4 new patients per month. The sample size was obtained using a prevalence of sickle cell anaemia of 2% and the sample size was calculated using Cochran sample size formula. Five milliliters (5ml) of venous blood sample was withdrawn from the peripheral vein in the upper limb of subjects using a standard venipuncture technique. The sample was rocked gently to mix and kept at room temperature and the haemostatic parameters (vWF, FVIII, D-dimer, L-arginine, fibrinogen, ADAMTS13) were assayed quantitatively with Bioassay Technozym kit using Microplate Reader (Labtech microplate auto ELISA plate reader, an IS0 13485:2003 CE and WHO compliance Co., Ltd. Shanghai International Holding Corp. GmbH; Europe) calibrated to a wavelength of 450 nm with strict adherence to the manufacturer's instructions, while PT and APTT were analysed with Fortress reagent and Uniscope SM801A Laboratory using water bath.Data management and statistical analyses were conducted using Statistical Analyses System SAS 9.4 (SAS Institute, Cary, North Carolina, USA) and p values less than .05were considered statistically significant. Results: The results showed the mean comparison of haemostatic parameters in sickle cell anaemia and control subjects. The comparison of haemostatic parameters showed significant(p<.05) increasesand decreases inVaso-Occlusive Crisis (VOC) and steady state respectively compared with the control group. There was statistically significantreduction in the mean comparison of L-Arginine (p<.01) in VOC) condition than steady state in relation to the control group in our study population, while D-Dimer, ADAMTS13 were also significantly reduced statistically (p<.01) in VOC condition than steady state compared with the control group. However, the mean FVIII inhibitor, Fibrinogen, PT (INR) and APTT were significantly higher (p<.01) in VOC than steady state when compared to controls with normal haemoglobin (HbAA).Correlations of haemostaticparameters by sickle cell anaemia subjects’ condition showed more significant positive correlations in VOC than steady state. Conclusion: This study showed a heightened hypercoagulability in Sickle Cell Snaemia(SCA)subjects, and further pave way for better understanding particularly the diagnostic variables underlying SCA, specific to each subject condition (steady state and VOC). Subjects with SCA, particularly during VOC, undergo significant haemostatic alterations that increase their risk of developing coagulation activation-related complications. Thus, though selected markers of coagulation were significantly different between the subject conditions, they were often significantly higher in the SCA.

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Section: Resultssupporting

confidence: 92%

Assessment of Some Haemostatic Parameters in Sickle Cell Anaemia Subjects Resident in Rivers and Bayelsa States

Mbeera

Akwuebu

Ezimah³

et al. 2021

JAMMR

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“…In a study of 6,814 individuals, FVIII levels were significantly higher in Black Americans than in White, Chinese, and Hispanic Americans (p<0.0001) [6]. FVIII levels are also higher in sickle cell, a prothrombotic disease, which is significantly more common amongst Blacks compared to Whites [7]. In contrast, the Factor V Leiden polymorphism and prothrombin gene G20210A…”

Section: Biologic Mechanismsmentioning

confidence: 98%

“…6 FVIII levels are also higher in sickle cell, a prothrombotic disease, which is significantly more common among Blacks compared with Whites. 7 In contrast, the factor V Leiden polymorphism and prothrombin gene G20210A variant are more prevalent among Northern and Southern Europeans, and are each associated with an increased odds of VTE (factor V Leiden VTE odds ratio [OR] 4.9; 1.1-7.3% minor allele frequency [MAF]; prothrombin gene G20210A VTE OR 3.1; 1.1-4.0% MAF, respectively). 8 Protein C and protein S gene mutations increase VTE odds among Chinese (OR 6.4; 0.04-1.2% MAF) and Japanese populations (OR 5.2; 0.9% MAF).…”

Section: Biologic Mechanismsmentioning

confidence: 99%

Racial and Ethnic Disparities in Cancer-Associated Thrombosis

et al. 2021

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Active malignancy increases the risk of developing venous thromboembolism (VTE) by four- to seven-fold. The risk of VTE, including deep vein thrombosis and pulmonary embolism, in patients with cancer varies based on several clinical factors, such as cancer stage and age. However, race and ethnicity are also associated with increased VTE risk. Black (African American) patients with cancer have a higher risk of developing VTE than White patients, while Asian/Pacific Islanders have a lower risk. Studies on cancer-associated thrombosis demonstrate a need to advance our understanding of both the biologic and sociologic underpinnings of the observed differences according to race. Addressing the causes of these disparities can better health outcomes for historically underserved patient populations.

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“…In contrast to Buseri et al [ 22 ], aPTT values provided by Nsiri et al [ 23 ] in Tunisia was 34.4 ± 4.2 seconds and was quite similar to the values obtained in this study among SCA subjects without CLU. An isolated elevation of factor VIII concentration has previously been reported in SCD patients, and it may explain the shortened aPTT [ 23 , 26 – 28 ]. The shortened aPTT indicates a highly active coagulation system and thus a hypercoagulable state which could lead to thrombosis; thrombosis could lead to venous congestion and subsequently venous hypertension which can facilitate the development and sustenance of a leg ulcer.…”

Section: Discussionmentioning

confidence: 99%

Enhanced Hypercoagulability in Sickle Cell Anaemia Patients with Chronic Leg Ulcers

Sackey

Dei‐Adomakoh

Olayemi

2020

Advances in Hematology

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Sickle Cell Anaemia (SCA) is associated with a hypercoagulable state resulting in a predisposition to venous thromboembolism. With improvements in the quality of care, more patients with SCA survive into adulthood with an associated increase in the frequency of end-organ damage and chronic complications such as chronic leg ulcers (CLUs). These ulcers rarely occur in the first decade of life and are recurrent, painful, and slow-to-heal. This study tested the hypothesis that coagulation is enhanced in SCA patients with CLU. 145 participants (50 SCA with CLU, 50 SCA without CLU, and 45 with haemoglobin AA) were assessed to determine their coagulation profile using selected tests of coagulation. The SCA with the CLU group had the lowest mean haemoglobin (Hb) concentration. SCA patients with and without CLUs had elevated mean platelet counts, shorter mean aPTT, and marginally prolonged mean PT compared to HbAA patients. SCA with CLUs patients had a significantly shortened aPTT than those without CLUs ( p = 0.035) and HbAA ( p = 0.009). There were significant differences in the mean PT between SCA with CLUs patients and HbAA ( p = 0.017); SCA without CLU and HbAA ( p = 0.014). SCA with and without CLUs patients had higher mean D-dimer levels compared to HbAA. There was a negative correlation between Hb concentration and duration of CLU (r = -0.331, p = 0.021). In conclusion, our study demonstrates a heightened hypercoagulability in SCA patients with CLUs. We did not test for platelet activation, and it is not clear what role, if any, the enhanced hypercoagulability plays in the pathogenesis of CLUs in SCA. It will be useful to ascertain if antiplatelet agents or/and anticoagulants quicken the healing of CLUs in SCA patients.

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Increased Level of Factor VIII and Physiological Inhibitors of Coagulation in Patients with Sickle Cell Disease

Cited by 7 publications

References 14 publications

Assessment of Some Haemostatic Parameters in Sickle Cell Anaemia Subjects Resident in Rivers and Bayelsa States

Assessment of Some Haemostatic Parameters in Sickle Cell Anaemia Subjects Resident in Rivers and Bayelsa States

Racial and Ethnic Disparities in Cancer-Associated Thrombosis

Enhanced Hypercoagulability in Sickle Cell Anaemia Patients with Chronic Leg Ulcers

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