Cytochemical Observations on β- Thalassaemia

Abstract

The periodic-acid Schiff (PAS) positive material in the normoblasts of homozygous [beta]-thalassaemia and related conditions was re-examined. The staining properties and mainly the degradation by diastase indicate that this PAS positive material is glycogen. Glycogen, Fe granules and Heinze body-like inclusion bodies may coexist in one cell. There is a rough positive correlation between severity of the disease and degree of PAS positivity. Adult cases of [beta] -thalassaemia trait have PAS negative normoblasts. The findings are discussed in the light of present concepts on the pathogenesis of [beta] -thalassaemia. Accumulation of glycogen may be a means of storing the energy which may occur in excess because of the severe reduction in hemoglobin synthesis.