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Cardiac iron overload in thalassemic patients: An endomyocardial biopsy study

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Abstract

Secondary heart failure induced by organ siderosis is the main cause of death in patients affected by thalassemia major. At present it cannot be predicted whether heart siderosis is correlated with iron overload and little is known about the real cardiac histological pattern of post transfusional hemochromatosis in patients with thalassemia major and intermedia. The study aim was to evaluate cardiac iron overload by non invasive and invasive techniques. Fifteen thalassemic patients were investigated and endomyocardial biopsy performed in ten revealed different grades of endomyocardial iron overload with histochemical positivity. Non invasive techniques are not able to furnish an exact picture of the cardiac hemochromatosis. There was a significant correlation between serum ferritin and myocardial iron grade.

Patients with elevated ferritin levels and poor compliance to chelating therapy are at high risk of severe heart hemochromatosis. It was seen that endomyocardial biopsy is a useful tool in studying myocardial iron.

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References

  1. Vullo C, Argiolu F, Borgatti L, De Sanctis V, Di Palma A, Gamberini MR, Ranalli F, Tanas R (1988) Il destino dei pazienti affetti da thalassemia major. Riv Ital Pediatr 14:533–564

    Google Scholar 

  2. Piomelli S (1989) Cooley's anemia management: 25 years of progress. In: Bucker CD, Gale RP, Lucarelli G (eds) Progress in clinical and biological research. Advances and controversies in thalassemia therapy. Liss, New York, pp 23–26

    Google Scholar 

  3. Lucarelli G, Weatherall DJ (1991) Clinical annotation. For debate: bone marrow transplantation for severe thalassemia. Br J Haematol 78:300–303

    PubMed  Google Scholar 

  4. Modell B, Berdoukas V (1984) The clinical approach to thalassemia. Grune and Stratton, London, p 161

    Google Scholar 

  5. Henry WL, Nienhuis AW, Wierner M, Miller DR, Canale VC, Piomelli S (1978) Echocardiographic abnormalities in patients with transfusion-dependent anemia and secondary myocardial iron deposition. Am J Med 64:547–553

    PubMed  Google Scholar 

  6. Cohen A (1978) Management of iron overload in the pediatric patient. Hematol Oncol Clin North Am 1:521–544

    Google Scholar 

  7. Mancuso L (1988) Problemi cardiologici nella thalassemia major. G Ital Cardiol 18:665–670

    PubMed  Google Scholar 

  8. Zurlo MG, De Stefano P, Borgna-Pignatti P, et al (1989) Survival and causes of death in thalassemia major. Lancet 1:27–30

    PubMed  Google Scholar 

  9. Wolfe L, Olivieri N, Sallan D, et al (1985) Prevention of cardiac disease by subcutaneous deferoxamine in patients with thalassemia major. N Engl Med 312:1600–1603

    Google Scholar 

  10. Weatherall DJ, Clegg JB (1981) Thalassemia syndrome, 3rd edn. Blackwell, Oxford

    Google Scholar 

  11. Scheuer PJ, Williams R, Muir AR (1962) Hepatic pathology in relatives of patients with haemochromatosis. J Pathol 84:53–56

    Google Scholar 

  12. Barosi G, Arbustini E, Gavazzi A, Grasso M, Pucci A (1989) Myocardial iron grading by endomyocardial biopsy. A clinico-pathologic study on iron overload patients. Eur J Haematol 42:382–388

    PubMed  Google Scholar 

  13. Modell B, Berdoukas V (1984) The clinical approach to thalassemia. Grune and Stratton, London, p 164

    Google Scholar 

  14. Olsen LJ, Edwards WD, Holmes DR Jr, Miller FA Jr, Nordstrom LA, Baldus WP (1989) Endomyocardial biopsy in hemochromatosis: clinico-pathologic correlates in six cases. J Am Coll Cardiol 13:116–120

    PubMed  Google Scholar 

  15. Fitchett DH, Coltart DJ, Littler WA, Leyland MJ, Trueman T, Gozzard DI, Peters TJ (1980) Cardiac involvement in secondary haemochromatosis: a catheter biopsy study and analysis of myocardium. Cardiovasc Res 14:719–724

    PubMed  Google Scholar 

  16. Buja LM, Roberts WC (1971) Iron in the heart. Etiology and clinical significance. Am J Med 51:209–221

    PubMed  Google Scholar 

  17. Mason JW, O'Connel JB (1989) Clinical merit of endomyocardial biopsy. Circulation 79:971–979

    PubMed  Google Scholar 

  18. Baandrup U, Olsen EGJ (1981) Critical analysis of endomyocardial biopsies from patients suspected of having cardiomyopathy. I. Morphological and morphometric aspects. Br Heart J 45:475–486

    PubMed  Google Scholar 

  19. Baandrup U, Florio RA, Rehahn M, Richardson PJ, Olsen ECJ (1981) Critical analysis of endomyocardial biopsies from patients suspected of having cardiomyopathy. II. Comparison of histology and clinical-haemodynamic information. 45:487–493

    Google Scholar 

  20. Short EM, Winkle RA, Billigham ME (1981) Myocardial involvement in idiopathic hemochromatosis and clinical improvement following venesection. Am J Med 70:1275–1279

    PubMed  Google Scholar 

  21. Folland E, et al (1979) Assessment of left ventricular ejection fraction and volumes by real-time two-dimensional echocardiography. Circulation 60:760

    PubMed  Google Scholar 

  22. Wyatt H, et al (1980) Cross-sectional echocardiography. II. Analysis of mathematic models for quantifying volume of formalin fixed left ventricle. Circulation 61:1119

    PubMed  Google Scholar 

  23. Wyatt H, et al (1978) Quantification of volumes in asymmetric left ventricles by 2-D echocardiography. Circulation [Suppl II] 58:730

    Google Scholar 

  24. Kremastinos DT, Toutouzas PK, Vyssoullis GP, Venetis CA, Vretou HP, Avgoustakis DG (1985) Global and segmental left ventricular function in beta-thalassemia. Cardiology 72:129–139

    PubMed  Google Scholar 

  25. Dimitrios TH, Kremastinos MD, Dimitrios Toutouzas MD, et al (1993) Left ventricular diastolic Doppler characteristics in β-thalassemia major. Circulation 88:1127–1135

    PubMed  Google Scholar 

  26. Kremastinos TD, Rentoukas E, Mavrogeni S, Kyriakides ZS, Politis C, Toutouzas P (1993) Left ventricular inflow pattern in beta-thalassemia major: a Doppler echocardiographic study. Eur Heart J 14:351–357

    PubMed  Google Scholar 

  27. Perrimond H, Chagnon C, Moulanier I, Guidicelli H, Bernard PJ (1991) The value of nuclear magnetic resonance in the study of iron overload in thalassemia patients. Ann Pediatr Paris 38:175–184

    PubMed  Google Scholar 

  28. Castriota Scanderberg A, Ferrozzi F, Butturini A, Izzi GC, Zavota L, Casa F, Giovannelli G (1990) Lo studio del sovraccarico di ferro in soggetti talassemici mediante risonanza magnetica nucleare. Riv Ital Pediatr 16:294–299

    Google Scholar 

  29. Johnston DL, Rice L, Vick GW, Hedrick TD, Rokey R (1989) Assessment of tissue iron overload by nuclear magnetic resonance imaging. Am J Med 87:40–47

    PubMed  Google Scholar 

  30. Celada A (1982) Iron overload in non-transfused patient with thalassaemia intermedia. Scand J Haematol 28:169–174

    PubMed  Google Scholar 

  31. Pippard MJ, Callender ST, Warner GT, Weatherall DJ (1979) Iron absorption in β-thalassaemia Intermedia. Lancet 2:819–821

    PubMed  Google Scholar 

  32. Borgna-Pignatti C, Castriota Scanderberg A (1991) Methods for evaluating iron stores and efficacy of chelation in transfusional hemosiderosis. Haematologica (Pavia) 76:409–413

    Google Scholar 

  33. Aldouri MA, Wonke B, Hoffbrand AV, Flynn DM, Ward SE, Agnew JE, Hilson AJ (1990) High incidence of cardiomyopathy in beta-thalassaemia patients receiving regular transfusion and iron chelation: reversal by intensified chelation. Acta Haematol 84:113–117

    PubMed  Google Scholar 

  34. Richard PJ (1974) King's endomyocardial bioptome. Lancet 1:660–664

    PubMed  Google Scholar 

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Lombardo, T., Tamburino, C., Bartoloni, G. et al. Cardiac iron overload in thalassemic patients: An endomyocardial biopsy study. Ann Hematol 71, 135–141 (1995). https://doi.org/10.1007/BF01702649

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  • DOI: https://doi.org/10.1007/BF01702649

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