Sickle cell variantsHemoglobin electrophoresis (< 2 months of age)Serial complete blood count, reticulocyte countHematologic studies (9 months of age)Parents' usual phenotypes
MCVHemoglobin A2 (%)§Hemoglobin F (%)§DNA dot blotOne parentOther parent
Homozygous sickle cell disease, or hemoglobin SS diseaseFSHemolysis and anemia usually by 6 to 12 months of ageNormal or increased<3.6< 25βS ASAS
Sickle β0-thalassemiaFSHemolysis and anemia usually by 6 to 12 months of ageDecreased>3.6< 25βAβS ASA on routine electrophoresis
A2 > 3.6 on quantitative electrophoresis
F increased on quantitative electrophoresis
MCV decreased on hemogram
Sickle β+-thalassemiaFSAMild anemia or no anemia by 2 years of ageNormal or decreased>3.6<25βAβS ASA on routine electrophoresis
A2 > 3.6 on quantitative electrophoresis
MCV decreased on hemogram
Hemoglobin SC diseaseFSCMild anemia or no anemia by 2 years of ageNormal or decreasedNot applicable<15βSβC ASAC