An Abnormal Substance Present in the Erythroblasts of Thalassaemia Major. Cytochemical Investigations

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Abstract
Studies of the reaction of human bone marrow cells, both normal and pathologic, to the periodic acid Shiff reaction (PAS) have shown that in 20 cases of thalassemia major a certain number of erythroblasts reacted positively. This was also observed with erythroblasts from the peripheral blood. Positivity is usually shown by bright red granules of varying form and size and an endo-cytoplasmatic distribution, and much less frequently in the form of a diffuse pink staining of the erythroblastic, and more rarely of the erythrocytic, cytoplasm. The substance which is responsible for the positivity of the PAS test was shown to be a gluco- or a muco-protein, or possibly a neutral mucopolysaccharide. This phenomenon can be considered one of the morphological and biological abnormalities of the "erythron" of patients affected by thalassemia major.

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