Which of the following can help distinguish the combination Hemoglobin S (Hb S) b0–thalassemia from sickle cell anemia?
a. Hematocrit
b. Hemoglobin electrophoresis
c. Ethnic background
d. Mean cell volume (MCV)
ANS: D
Hb S is a structurally abnormal b globin chain; this gene is inherited from one parent. The b0 gene is inherited from the other parent, which means that no b chain production occurs from that gene. Thus the patient will not have any normal b chains to combine with a chains and form Hb A. The patient will have primarily Hb S, with an elevated Hb F, which is the same pattern seen in homozygous Hb S (i.e., sickle cell anemia). However, in sickle cell anemia the red cells are normocytic, whereas in Hb S b0–thalassemia the red cells will be microcytic.
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