Biophysical changes of red cells with thalassemia-like abnormal hemoglobin

Hemoglobin (Hb) E and Hb Constant Spring (CS) are known to have thalassemia-like effects. Investigation on the red cell physical changes in these two abnormalities has been done to clarify the diversity of red cell changes between thalassemia and thalassemia-like abnormal hemoglobin (Hb E represents beta-thalassemia and Hb CS represents alpha-thalassemia). Eleven cases with homozygous Hb CS (CS/CS), 7 homozygous Hb E subjects (E/E) and one double heterozygous case with Hb CS and Hb E were included in this study. The red cells were analyzed by the H* 1 hematology analyzer. The E/E red cells had significantly smaller MCV than the CS/CS red cells (p < 0.001). The smaller MCV of E/E red cells was attributed to markedly increased percent microcyte (p < 0.001) and significantly lower percent macrocyte (p < 0.001) as compared to CS/CS red cells. Degree of heterogeneity in cell volume as indicated by red cell distribution width (RDW) was not significantly different between the two abnormal hemoglobin containing red cells. It was also noted that the CS/CS cases had a more significant increase in small RBC than the E/E cases (p < 0.001). Significant lower intraerythrocyte hemoglobin concentration values of the CS/CS red cells as compared to E/E red cells were shown: cellular hemoglobin concentration mean (CHCM) (p < 0.001), percent hyperchromic red cell (p < 0.001) and hemoglobin distribution width (HDW) (p = 0.0367). Higher values for the CS/CS red cells were MCH (p < 0.001) and percent hypochromic red cells (p < 0.001). Red cells from both genotypes had significant decreases in red cell deformability.(ABSTRACT TRUNCATED AT 250 WORDS)