Genotype variation

All genotypes of sickle cell disease have similar manifestations, although the frequency and severity are usually greater in homozygous sickle cell disease and sickle cell-b°-thalassemia than in sickle cell-hemoglobin C disease and sickle cell-b +-thalassemia. The only exception to this pattern is retinal disease which is more severe in the latter two conditions. The clinical complications described below apply principally to homozygous sickle cell disease and to a lesser extent to other genotypes.

Hemolysis and low levels of vaso-occlusion are continuous features of sickle cell disease even when the patient is clinically well (steady state), but the disease is also punctuated by a variety of acute events commonly called crises. Crisis is an overused term in sickle cell disease and should never be used without an adjective describing its nature.

Common acute clinical problems encountered in the management of sickle cell disease include the aplastic crisis, acute splenic sequestration, septicemia, stroke, painful crisis, acute chest syndrome, pregnancy, and postoperative management.

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