Hypercoagulability in Sickle Cell Disease: New Approaches to an Old Problem

Abstract

Patients with sickle cell disease (SCD) exhibit high plasma levels of markers of thrombin generation, depletion of natural anticoagulant proteins, abnormal activation of the fibrinolytic system, and increased tissue factor expression, even in the non-crisis steady state. In addition, platelets and other cellular elements are chronically activated in the non-crisis state. Despite an abundance of evidence for coagulation and platelet activation, it remains uncertain whether these changes contribute to the pathophysiology of SCD or are, rather, simple epiphenomena. With the occurrence of macrovascular thrombotic complications in SCD, as well as the recognition that soluble CD40 ligand is biologically active in SCD, coagulation and platelet activation may indeed play a role in SCD pathophysiology. Defining a role for hypercoagulability in SCD requires further understanding of its pathogenesis. Furthermore, the conduct of well-controlled clinical trials using anticoagulants and antiplatelet agents and using a variety of clinical endpoints is warranted.

The Outcome of Preoperative Transfusion Guideline on Sickle Cell Disease Patients at King Fahd Hospital-Jeddah (KSA)

“…For this reason, heparin could be a useful drug for management of the disease in standard care and also in postoperative settings, where natural and necessary coagulative responses could cascade into detrimental levels of coagulation [22]. However use of heparin was not risk free as it could lead to greater levels of bleeding post operatively.…”

“…For this reason, heparin might be used for management of the disease for standard care and also in post-operative settings, where natural and necessary coagulative responses could cascade into detrimental levels of coagulation factors [22]. There is some risk involved in the use of heparin following surgery as it can aggravate bleeding, however, with proper monitoring and care these risks could be easily mitigated in hospital settings [22].…”

“…Sickle cell disease was considered as a hyper-coagulation state, even when there was no obvious history of injury or tear to trigger such a coagulation pathway [22,32]. For this reason, heparin could be a useful drug for management of the disease in standard care and also in postoperative settings, where natural and necessary coagulative responses could cascade into detrimental levels of coagulation [22].…”

“…However use of heparin was not risk free as it could lead to greater levels of bleeding post operatively. But with proper management and care these risks could easily be mitigated in hospital settings [22].…”

The Role of Hu in the Management of Vaso-Occlusive Crises in Sickle Cell Anemia.

“…The latter is characterized by severe acute pain in the abdomen and /or extremities, and can result in organ damage as result of progressive vasculopathy and acute infarctions. (13)(14)(15) The treatment of SCA can vary between countries according to the available resources and disease prevalence. Treatment options include rehydration and blood transfusion to correct anemia, vaccinations and prophylactic antibiotics to reduce and prevent infections, as well as pain management to reduce the burden of painful crisis and improve the functioning abilities of patients.…”