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Thalassemia: Pathophysiology, Clinical and Laboratory Findings

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Radiology of Thalassemia

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Abstract

Thalassemias are a heterogeneous group of disorders of hemoglobin (Hb) synthesis, which have in common a reduced rate of synthesis of one or more of the globin chains of normal human Hb [25]. This leads to a variable degree of premature cell destruction, ineffective erythropoiesis and/or hemolysis, and, finally, a variable degree of anemia.

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References

  1. Bank A, Marks PA (1966) Excess a-chain synthesis relative to ß-chain synthesis in thalassemia major and minor. Nature 212: 1198.

    Article  PubMed  CAS  Google Scholar 

  2. Bargellesis A, Pontremoli S, Menic C, Conconi F (1968) Excess of alpha globin synthesis in homozygous beta-thalassemia and its removal from the red cell cytoplasm. Eur J Biochem 3: 364

    Article  Google Scholar 

  3. Costin G, Cocut MD, Human CB, Ortega JA (1977) Endocrine abnormalities in thalassemia major. Am J Dis Child 133: 497

    Google Scholar 

  4. Donabedia RK, Felig P, Genel M (1974) Endocrine evaluation in thalassemia major. Ann NY Acad Sci 232: 226

    Article  Google Scholar 

  5. Dormer P, Betke K (1978) Erythroblasts kinetics in homozygous and heterozygous beta-thalassemia. Br J Haemat 38: 5

    Article  CAS  Google Scholar 

  6. Engle MA (1964) Cardiac involvement in Cooley’s anemia. Ann NY Acad Sci 119: 694

    Article  PubMed  CAS  Google Scholar 

  7. Fessas P (1963) Inclusions of hemoglobin in erythroblasts and erythrocytes of thalassemia. Blood 21: 21

    PubMed  CAS  Google Scholar 

  8. Fessas P, Loukopoulos D (1974) Theß-thalassemia. Clin Haematol 3: 411

    Google Scholar 

  9. Flynn DM, Fairney A, Jackson D, Cayton BA (1976) Hormonal changes in thalassemia major. Arch Dis Child 51: 828

    Article  PubMed  CAS  Google Scholar 

  10. Gabuzda TG, Nathan DG, Gardner FH (1963) The turnover of hemoglobins A, F and A2 in the peripheral blood of three patients with thalassemia. J Clin Invest 42: 1678

    Article  PubMed  CAS  Google Scholar 

  11. Gabuzda TG (1966) Hemoglobin H and the red cell. Blood 27: 567

    Google Scholar 

  12. Hammond GD, Ishikawa A, Keighly G (1962) Relationship between erythropoietin and severe anemia in hypoplastic and hemolytic states. In: Jacobson LO, Doyle M (eds) Erythropoiesis. Grune and Stratton, New York

    Google Scholar 

  13. Jacob HS (1970) Mechanism of Heinz body formation and attachment to red cell membrane. Semin Hematol 7: 341

    PubMed  CAS  Google Scholar 

  14. Jandl JH, Greenberg MS (1959) Bone marrow failure due to relative nutritional deficiency in Cooley’s hemolytic anemia. New Engl J Med 266: 461

    Article  Google Scholar 

  15. Kaplan E, Zuelzer WW (1950) Erythrocyte survival studies in childhood. II. Studies in Mediterranean anaemia. J Lab Clin Med 36: 517

    PubMed  CAS  Google Scholar 

  16. Kremastinos DT, Toutouzas PK, Vyssoulis GP, Venetis CA, Avgoustakis DG (1984) Iron overload and left ventricular performance inß-thalassemia. Acta Cardiol (Brux) 39: 29

    CAS  Google Scholar 

  17. Livadas DP, Sofroniadou K, Souvatzoglou A, Boukis MA, Siafaka L, Koutras DA (1984) Functional insufficiency of thyroid and pituitary gland in beta-homozygous thalassemia with hemosíderosis. Acta Endorinol (Suppl) (Copenh) 265: 43

    CAS  Google Scholar 

  18. Loukopoulos D, Fessas P (1965) The distribution of hemoglobin types in thalassemic erythrocytes. J Clin Invest 44: 231

    Article  PubMed  CAS  Google Scholar 

  19. Nathan DG, Gunn RB (1966) Thalassemia: the consequence of unbalanced hemoglobin synthesis. Am J Med 41: 815

    Article  PubMed  CAS  Google Scholar 

  20. Nathan DG, Stossel TB, Gunn RB, Zarkowsky HS, Laforet MT (1969) Influence of hemoglobin precipitation on erythrocyte metabolism in alpha and beta thalassemia. J Clin Invest 48: 33

    Article  PubMed  CAS  Google Scholar 

  21. Rachmilewitz EA and Thorell B (1972) Hemochromes in single inclusion bodies in red cells of beta thalassemia. Blood 39: 794

    PubMed  CAS  Google Scholar 

  22. Rachmilewitz EA (1976) The role of intracellular hemoglobin precipitation, low MCHC and iron overload on red blood cell membrane peroxidation in thalassemia. Birth defects 12: 123

    PubMed  CAS  Google Scholar 

  23. Wainscoat JS, Kanavakis E, Wood WG, Letsky DJ, Huehns ER, Marsh GW, Higgs DR, Clegg JB, Weatherall DJ (1983) Thalassemia intermedia in Cyprus. The interactions of a and β thalassemia. Br J Haematol 53: 411

    Article  PubMed  CAS  Google Scholar 

  24. Weatherall DJ, Clegg JB, Naughton MA (1966) Globin synthesis in thalassemia. Nature 212: 1198

    Article  Google Scholar 

  25. Weatherall DJ, Clegg JB (1981) The thalassemia syndromes. Blackwell Scientific, Oxford

    Google Scholar 

  26. Wickramasinghe SN, Letsky E, Moffatt (1973) Effect of a chain precipitates on bone marrow function in homozygousβthalassemia. Br J Haematol 25: 123

    Article  CAS  Google Scholar 

  27. Williams WJ, Beutler E, Erslev AJ, Lichtman MA (1983) Hematology. McGraw-Hill, New York

    Google Scholar 

  28. Wintrobe MM (1981) Clinical hematology. Lea and Febiger, Philadelphia

    Google Scholar 

  29. Zaino EE (1980) Pathophysiology of thalassemia. Ann NY Acad Sci 344: 284

    Article  PubMed  CAS  Google Scholar 

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Authors
  1. A. Fertakis
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Editors and Affiliations

  1. Department of Radiology, University of Athens, School of Medicine, Areteion Hospital, 115 28, Athens, Greece

    Constantin Papavasiliou  & Theophanis Cambouris  & 

  2. Department of Internal Medicine, University of Athens, School of Medicine, 115 28, Athens, Greece

    Phaedon Fessas

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© 1989 Springer-Verlag Berlin Heidelberg

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Fertakis, A. (1989). Thalassemia: Pathophysiology, Clinical and Laboratory Findings. In: Papavasiliou, C., Cambouris, T., Fessas, P. (eds) Radiology of Thalassemia. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-72587-6_3

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  • DOI: https://doi.org/10.1007/978-3-642-72587-6_3

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-72589-0

  • Online ISBN: 978-3-642-72587-6

  • eBook Packages: Springer Book Archive

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