Sickle Pathogenesis

Bunn HF Pathogenesis and treatment of sickle cell disease. N Engl J Med 1997 337 762. 

Other factors may be responsible for the pathogenesis of some of the clinical features of SCD. Sickle cells can obstruct blood flow to the spleen, leading to functional asplenia, which is loss of splenic function despite having an intact spleen. These patients... 

The neovascular lesions of talc retinopathy are thought to be associated with peripheral arteriolar nonperfusion, which leads to retinal ischemia and secondary neovascularization. Such a pathogenesis is quite similar to that seen in sickle cell retinopathy and is confirmed by the predominantly supertemporal location of the neovascular proliferation. Macular fibrosis with significant visual loss has also been associated with talc retinopathy. 

Factors in addition to sickling may be responsible for the pathogenesis of a number of the clinical manifestations associated with SCD. Obstruction of blood flow to the spleen by sickle cells can result in functional asplenia, defined as the loss of splenic function with an intact spleen. These patients may also have deficient opsonization. Impaired splenic function increases susceptibility to infection by encapsulated organisms, particularly pneumococcal disease. Coagulation abnormalities in SCD may be the result of continuous activation of the hemostatic system or disorganization of the membrane layer. " ... 

Singer K. 1951. The pathogenesis of sickle cell anemia A review. Am. J. Clin. Pathol. 21, 858. 

Table 3-2. Pathogenesis of sickle cell anemia Dominant autosomal gene...

Knowledge of the molecular alteration in sickle cell anemia has contributed to an understanding of the pathogenesis of the disease and has also generated some hope for therapy. Two compounds have been used in the treatment of sickle cell anemia urea and cyanate. 

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