ABSTRACT
Hereditary persistence of fetal hemoglobin (HPFH) is a benign condition in which significant fetal hemoglobin production continues well into adulthood, disregarding the normal shutoff point after which only adult-type hemoglobin should be produced. The percentage of incorrect expression might be as low as 10%-15% or as high as 100% of the total hemoglobin, usually higher in homozygotes than in heterozygotes. The present case is a typical example of homozygous HPFH.
ABSTRACT
PURPOSE OF STUDY: Study was aimed to observe benefits of transfusion of neocytes/pooled neocytes over packed RBCs by super transfusion regimen in ß-thalassemic patients and its comparison with related studies. MATERIALS AND METHODS: Twelve cases were selected and were equally divided into control and study group in which packed RBCs and neocytes were transfused respectively for one year. In study group, average 8 transfusions were required over 14 in control group. RESULTS: A marked extension of transfusion interval was observed in study group patients receiving neocytes transfusion (avg. 45.8 days; range, 32-54) over control group patients i.e. those receiving conventional packed RBCs (avg. 26.1; range 22-30). There is an average 40.8% reduction in the volume of RBCs required in the study group compared to control group and this difference is statistically significant (p = 0.00075). In study group, 20.1% reduction in iron overload and increase of iron by 9.6% in control group was reported. CONCLUSION: We observed that neocytes transfusion is effective in extending the transfusion interval as well as in reducing the blood requirement and iron over load.
