Hematological data analysis in children with thalassemia trait or hemoglobin H disease in Taiwan
Yang, C.P.; Hung, I.J.
Journal of the Formosan Medical Association 90(6): 591-597; 586
1991
ISSN/ISBN: 0929-6646 PMID: 1681007 Document Number: 379116
In the past several years, we have collected 91 cases diagnosed as .beta.-thalassemia trait, 94 cases as .alpha.-thalassemia trait and 43 cases as hemoglobin H disease. The ages ranged from 6 months to 15 years. The hematological data were analysed. We calculated the mean .+-. SD for each item of the erythrocyte indices (RBC, Hgb, Hct, MCV, MCH, MCHC .+-. RDW) and the Hb electrophoretic results (Hb A, Hb A2, Hb F, Hb H and Hb Bart's) in each group, and tabulated them. Then, comparisons were made between .beta. and .alpha.-thalassemia traits or between the thalassemia trait and hemoglobin H disease. We found that each of the mean values for Hgb, Hct, MCV and MCH for children with the .beta.- thalassemia trait was significantly lower than that for children with .alpha.-thalassemia trait. The mean MCV value of the .beta.-thalassemia trait group was 60.7 .+-. 3.4 fl, and 87% of the MCV values in this group ranged from 55 to 65 fl. The mean MCV value for the .alpha.-thalassemia trait group was 63.3 .+-. 3.3 fl, and 56% of the MCV values in this group ranged from 60-65 fl. The MCV values for all of the 185 children studied with either the .beta.- or .alpha.-thalassemia trait ranged from 54 to 71 fl, and were far below the normal ranges for MCV values for each age-specific group. The mean percentage of deviation of MCV from normal was greater than that of Hgb in both the .beta.- and .alpha.-thalassemia trait groups. A comparison of those with .beta.-thalassemia trait 6 years old under those over 6 years showed that both the mean values of RBC and Hgb were higher in the older Subgroup, while the mean MCV values did not show any significant differences between these 2 subgroups. The mean MCV and Hgb values were higher in the older subgroup (> 6 yrs) with the .alpha.-thalassemia trait. In the .beta.-thalessemia trait group, the mean Hb A2 value was 5.64 .+-. 1.20%, 80% of thos with the .beta.-thalassemia trait 4 years old and under showed Hb F component by cellulose acetate electrophoresis, while only 27% of the children older than 4 years showed this component. The mean MCHC value in the Hg H disease group was lower than that in the whole thalassemia trait group; while RDW values were somewhat higher in children with Hb H disease than those in the thalassemia trait groups. The mean value of Hb H in the Hb H disease group was 4.86 .+-. 4.45%. Fifty-six percent of the children in this group showed Hb Bart's on Hb electrophoresis (5.60 .+-. 3.25%).