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Gangliosides, sialoglycoproteins and glucocerebroside in the spleen and bone marrow of patients with β-thalassemia major

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Ricerca in clinica e in laboratorio

Summary

Bone marrow and spleen samples were studied biochemically to elucidate the nature of the material stored in the large histiocytes present in β-thalassemic patients. Only the protein-bound N-acetylneuraminic acid (NANA) content of the spleen of homozygous β-thalassemic patients was significantly higher than that of controls. In the bone marrow, which contained a large number of thalassemic storage cells, the lipid-bound NANA and the glucocerebroside showed a moderate increase, while the amount of protein-bound NANA was much greater than that in controls. These results, in agreement with ultrastructural and histochemical observations, differentiate thalasseminc storage cells from other storage cells found in diseases characterized by an increased destruction of blood cells and suggest that there may also be an impairment of the sialoglycoprotein metabolism of the red blood cells in homozygous β-thalassemia.

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Authors and Affiliations

  1. Istituto di Anatomia ed Istologia Patologica dell'Università degli Studi di Ferrara, Via Fossato di Morlara 66, 44100, Ferrara, Italy

    Guidalberto Fabris, Italo Bearzi & Carlo Alberto Beltrami

  2. Cattedra di Biochimica, Facoltà di Farmacia dell'Università degli Studi di Milano, Milano, Italy

    Donatella Primi, Silvia Di Palma & Bruno Berra

Authors
  1. Guidalberto Fabris
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  2. Italo Bearzi
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  3. Carlo Alberto Beltrami
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  4. Donatella Primi
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  5. Silvia Di Palma
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  6. Bruno Berra
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Fabris, G., Bearzi, I., Beltrami, C.A. et al. Gangliosides, sialoglycoproteins and glucocerebroside in the spleen and bone marrow of patients with β-thalassemia major. La Ricerca Clin. Lab. 8, 148–157 (1978). https://doi.org/10.1007/BF02904987

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  • DOI: https://doi.org/10.1007/BF02904987

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