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Much basic information about sickle-cell disease has been acquired in recent years, but some aspects remain unclarified. It might be useful to review and discuss briefly certain facts and speculations which concern this hereditary blood dyscrasia of Negroes. An article presenting its conjunctival manifestations is presented elsewhere in this issue.
One fundamental defect of the disease has been established—a genetically determined alteration in amino acid composition of the hemoglobin molecule. Under certain conditions of oxygen tension and pH, red cells containing this hemoglobin form a characteristic sickle shape. Hemolysis and thrombosis are known to be prominent factors in the varied clinical pathology of sickle-cell disease. Undoubtedly, sludging of blood flow is another important factor in the pathogenesis of clinical signs. The conjunctival vascular bed with its readily examined vascular stasis may be of assistance in future studies of the pathophysiology of sickle-cell disease—for it seems a reasonable assumption that the
Paton D. Background to Sickling. Arch Ophthalmol. 1961;66(1):3-5. doi:10.1001/archopht.1961.00960010005003