PubMed · 15658441
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Junko Murakami. 2004. [Abnormal Hb (hemoglobin)].. https://pubmed.ncbi.nlm.nih.gov/15658441/
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This article summarizes my presentation at the Pediatric Hematology-Oncology Symposium in honor of Dr. Stephen A. Feig in April 2005. Areas highlighted reflect key topics whose evolution parallel and were impacted by Steve's exceptional academic career. It is by no means an exhaustive summation of all advances in the biology and treatment of sickle cell disease. The interested reader is encouraged to consider a number of well written recently published reviews that cover additional areas of scientific and medical advancement in the pathophysiology and care of sickle cell disease 1-5.
PURPOSE OF REVIEW: This article discusses the importance of leukocyte adhesion in sickle cell disease, and how this could be modulated for clinical benefit. RECENT FINDINGS: Recurrent inflammation and vasculopathy occur in sickle cell disease. As a result, leukocytes and vascular endothelial cells are activated and increase their expression of adhesion molecules. Adhesion of leukocytes to other blood cells and endothelium contributes to vaso-occlusion in sickle cell disease. High-level expression of adhesion molecules by leukocytes is associated with clinically severe disease. Pancellular membrane lipid abnormalities, including reduced proportions of omega-3 fatty acids, occur in sickle cell disease. These lipid abnormalities are more severe in patients with disease complications and in those with a greater degree of anaemia. Since lipid constitution of cell membranes affects surface expression of adhesion molecules, the above findings could account for earlier observations that omega-3 fatty acids reduce P-selectin expression and reduce the frequency of sickle cell crisis. By inhibition of nuclear factor kappaB, glucocorticoids reduce activation of vascular endothelial cells, their expression of ligands for leukocyte adhesion molecules, and vaso-occlusion. Monoclonal antibodies to vascular endothelial intercellular adhesion molecule-1 inhibited hypoxia-induced vaso-occlusion in transgenic sickle mice. SUMMARY: Although hydroxyurea and glucocorticoids reduce adhesion molecule expression by leukocytes and vascular endothelial cells, cytotoxicity and systemic side effects dampen enthusiasm for their use in sickle cell disease. Omega-3 fatty acids have shown promising efficacy and safety in pilot studies. A large clinical trial of these naturally occurring substances is required.
A 9-year-old boy, originally from Zaire, presented with homozygous sickle-cell disease and a unilateral avascular necrosis of the femoral head. He was treated with a triple pelvic osteotomy to improve containment and stimulate remodelling of the head following prior partial reduction in a spica cast. Four and a half years postoperatively the patient was pain free with normal hip function. Radiography revealed a well-centred hip with a mild coxa magna and a nearly spherical femoral head. In geographic regions with a small black population, sickle cell disease and related orthopaedic problems are rarely encountered, but one must be prepared for the occasional patient. Triple pelvic osteotomy for the treatment of femoral head necrosis associated with sickle cell disease has not yet been reported and has led in this case to a highly satisfying improvement of the hip.