Abstract
Sickle cell disease is an autosomal recessive genetic disease. Vaso occlusive crisis (VOC) is frequently seen in such patients. Painful VOC is usually recurrent, of variable severity due to many factors and its management poses important challenge in the clinical practice. Few patients do not respond to standard therapies and continue to suffer severe pain for prolonged period or land to serious life threatening situation. The red cell exchange by aphaeresis is presumed to be one efficient alternative in this situation which can reduce the level of HbS below 40% - 50%. However, it is costly and not available everywhere. Both circumstances are common in our state where incidence of sickle cell disease is quite high. In such situations simple red cell exchange i.e. removing 1 unit (350 ml) of blood manually (by phlebotomy) and replacement with one unit normal red cell is effective. All of our four cases of SCA with severe acute VOC, are not responding to standard therapy but responded efficiently to this simple red cell exchange transfusion. Our present observation may pave the way of one simple, affordable, and effective measure to reduce the pain of severe acute VOC not responding to standard therapy. Moderate reduction of HbS by 8% - 14% by simple red cell exchange transfusion was associated with relief of pain of acute VOC; a new observation was reported in all our 4 cases which need to be validated by larger controlled studies.
Highlights
Homozygous sickle cell disease (SCD) is an autosomal recessive genetic disease
Vaso-occlusion is the outcome of dynamic combination of abnormalities in haemoglobin structure and function, red cell membrane integrity, erythrocyte density, endothelial activation micro vascular tone, inflammatory mediators and coagulation
We report four cases of SCD with severe Vaso occlusive crisis (VOC) not responding to standard therapy which were managed successfully with simple red cell exchange transfusion
Summary
Homozygous sickle cell disease (SCD) is an autosomal recessive genetic disease. The fundamental defect in SCD is the substitution of thymine for adenine in the sixth codon of the gene for the β-globin chain, leading to a replacement of glutamic acid by valine at this site. Red cell apheresis is the most efficient method to reduce the HbS level immediately without increasing the viscosity and successfully tried in patients with severe VOC not responding to standard therapies. This Apheresis procedure is expensive and it needs specialized equipment and set up. Simple exchange red cell transfusion though less effective than red cell apheresis can reduce HbS level and has not been studied properly in this context. Red cell exchange can provide needed oxygen carrying capacity while reducing the overall viscosity of blood. We report four cases of SCD (homozygous) with severe VOC not responding to standard therapy which were managed successfully with simple red cell exchange transfusion
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