Abstract
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.
Highlights
Sickle Cell Anaemia (SCA) is associated with an increase in prothrombotic factors and a decrease in physiologic anticoagulants, resulting in a predisposition to venous thromboembolism [1]
Post hoc analysis showed a significant difference in mean Hb concentration between the SCA with chronic leg ulcers (CLUs) and SCA without CLU groups (p 0.038), and that SCA without CLU participants were younger than SCA with CLU and HbAA participants (p 0.003), Table 1
SCA patients with and without CLU had elevated mean platelet counts, shorter mean activated partial thromboplastin time (aPTT), marginally prolonged mean Prothrombin time (PT) compared to HbAA participants
Summary
Sickle Cell Anaemia (SCA) is associated with an increase in prothrombotic factors and a decrease in physiologic anticoagulants, resulting in a predisposition to venous thromboembolism [1]. 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 leg ulcers [2]. SCA patients may develop recurrent, painful, slow-to-heal chronic leg ulcers (CLU). Due to the chronic nature of these ulcers, SCA patients with CLUs may experience significant disfigurement, social isolation, and loss of income [3]. E increased disability, absence from work or school, and high utilization of healthcare resources severely affect the quality of life of individuals with SCA and CLUs [8, 9]. Ankra-Badu (1992) reported the mean duration of a sickle cell CLU in Ghana as three and half years [10], a more recent study obtained a mean duration of 4.6 years [5]. Ankra-Badu (1992) reported the mean duration of a sickle cell CLU in Ghana as three and half years [10], a more recent study obtained a mean duration of 4.6 years [5]. is is similar to what is obtained in the United States [11]
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