Skip to main content
SpringerLink
Log in

A national registry of haemoglobinopathies in Greece: Deducted demographics, trends in mortality and affected births

  • Original Article
  • Published:
Annals of Hematology Aims and scope Submit manuscript

Abstract

Haemoglobinopathies are the most common hereditary disorders in Greece. Although there is a successful national prevention program, established 35 years ago, there is lack of an official registry and collection of epidemiological data for haemoglobinopathies. This paper reports the results of the first National Registry for Haemoglobinopathies in Greece (NRHG), recently organized by the Greek Society of Haematology. NRHG records all patients affected by thalassaemia major (TM), thalassaemia intermedia (TI), "H" Haemoglobinopathy (HH) and sickle cell disease (SCD). Moreover, data about the annual rate of new affected births along with deaths, between 2000 and 2010, are reported. A total of 4,506 patients are registered all over the country while the number of affected newborns was significantly decreased during the last 3 years. Main causes for still having affected births are: (1) lack of medical care due to financial reasons or low educational level; (2) unawareness of time limitations for prenatal diagnosis (PD); due either to obstetricians’ malpractice or to delayed demand of medical care of couples at risk; and (3) religious, social or bioethical reasons. Cardiac and liver disorders consist main causes for deaths while life expectancy of patients lengthened after 2005 (p < 0.01). The NRHG of patients affected by haemoglobinopathies in Greece provides useful data about the haemoglobinopathies in the Greek population and confirms the efficacy of the National Thalassaemia Prevention Program on impressively decreasing the incidence of TM and sickle cell syndromes.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

References

  1. Schizas N, Tegos C, Voutsadakis A, Arabatzis G, Angelopoulou P, Chrysanthopoulos K, Athanasiadou A, Bathrelou S, Rombos J, Skarlos D, Davakis M (1977) The frequency and distribution of β-thalassaemia and abnormal hemoglobins in Greece. A study of 15.000 reruits. Hell Armed ForcesMed Rev 11(suppl 1):197–203

    Google Scholar 

  2. Angastiniotis M, Modell B, Englezos P, Boulyjenkov V (1995) Prevention and control of haemoglobinopathies. Bull World Health Organ 73:375–386

    PubMed  CAS  Google Scholar 

  3. Cao A, Rosatelli C, Galanello R (1991) Population-based genetic screening. Curr Opin Genet Dev 1:48–53

    Article  PubMed  CAS  Google Scholar 

  4. Scalone L, Mantovani LG, Krol M, Rofail D, Ravera S, Bisconte MG, Borgna-Pignatti C, Borsellino Z, Cianciulli P, Gallisai D, Prossomariti L, Stefàno I, Cappellini MD (2008) Costs, quality of life, treatment satisfaction and compliance in patients with beta-thalassaemia major undergoing iron chelation therapy: the ITHACA study. Curr Med Res Opin 24(7):1905–1917

    Article  PubMed  Google Scholar 

  5. Pennell DJ, Berdoukas V, Karagiorga M, Ladis V, Piga A, Aessopos A, Gotsis ED, Tanner MA, Smith GC, Westwood MA, Wonke B, Galanello R (2006) Randomized controlled trial of deferiprone or deferoxamine in beta-thalassaemia major patients with asymptomatic myocardial siderosis. Blood 107:3738–3744

    Article  PubMed  CAS  Google Scholar 

  6. Westwood MA, Firmin DN, Gildo M, Renzo G, Stathis G, Markissia K, Vasili B, Pennell DJ (2005) Intercentre reproducibility of magnetic resonance T2* measurements of myocardial iron in thalassaemia. Int J Cardiovasc Imaging 21:531–538

    Article  PubMed  Google Scholar 

  7. Chouliaras G, Berdoukas V, Ladis V, Kattamis A, Chatziliami A, Fragodimitri C, Karabatsos F, Youssef J, Karagiorga-Lagana M (2011) Impact of magnetic resonance imaging on cardiac mortality in thalassaemia major. J Magn Reson Imaging 34:565–569

    Article  Google Scholar 

  8. Modell B, Khan M, Darlison M, Westwood MA, Ingram D, Pennell DJ (2008) Improved survival of thalassaemia major in the UK and relation to T2* cardiovascular magnetic resonance. J Cardiovasc Magn Reson 10:42

    Article  PubMed  Google Scholar 

  9. Modell B, Khan M, Darlison M (2000) Survival in beta-thalassaemia major in the UK: data from the UK Thalassaemia Register. Lancet 355:2051–2052

    Article  PubMed  CAS  Google Scholar 

  10. Telfer P, Coen GP, Christou S, Hadjigavriel M, Kolnakou A, Pangalou E, Pavlides N, Simamonian K, Skordos G, Sitarou M, Angastiniotis M (2006) Survival of medically treated thalassaemia patients in Cyprus. Trends and risk factors over the period 1980–2004. Haematol 91:1187–1192

    Google Scholar 

  11. Borgna-Pignati C, Rugolotto S, Zhao H, Cappellini MD, Del Vecchio GC, Romeo MA, Forni GL, Gamberini MR, Ghilardi R, Piga A, Cnaan A (2004) Survival and complications in patients with thalassaemia major treated with transfusion and deferoxamine. Haematol 89:1187–1193

    Google Scholar 

  12. Ladis V, Chouliaras G, Berdousi H, Kanavakis E, Kattamis C (2005) Longitudinal study of survival and causes of death in patients with thalassaemia major in Greece. Ann N Y Acad Sci 1054:445–450

    Article  PubMed  Google Scholar 

  13. Charafeddine K, Isma'eel H, Charafeddine M, Inati A, Koussa S, Naja M, Taher A (2008) Survival and complications of beta-thalassaemia in Lebanon: a decade's experience of centralized care. Acta Haematol 120(2):112–116

    Article  PubMed  CAS  Google Scholar 

  14. Bazrgar M, Peiravian F, Abedpour F, Karimi M (2011) Causes for hospitalization and death in Iranian patients with β-thalassaemia major. Pediatr Hematol Oncol 28(2):134–139

    Article  PubMed  Google Scholar 

  15. Ladis V, Chouliaras G, Berdoukas V, Chatziliami A, Fragodimitri C, Karabatsos F, Youssef J, Kattamis A, Karagiorga-Lagana M (2011) Survival in a large cohort of Greek patients with transfusion-dependent beta thalassaemia and mortality ratios compared to the general population. Eur J Haematol 86:332–338

    Article  PubMed  Google Scholar 

  16. Voskaridou E, Christoulas D, Bilalis A, Plata E, Varvagiannis K, Stamatopoulos G, Sinopoulou K, Balassopoulou A, Loukopoulos D, Terpos E (2010) The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS). Blood 115:2354–2363

    Article  PubMed  CAS  Google Scholar 

  17. Angelucci E, Muretto P, Nicolucci A, Baronciani D, Erer B, Gaziev J, Ripalti M, Sodani P, Tomassoni S, Visani G, Lucarelli G (2002) Effects of iron overload and hepatitis C virus positivity in determining progression of liver fibrosis in thalassaemia following bone marrow transplantation. Blood 100(1):17–21

    Article  PubMed  CAS  Google Scholar 

  18. Borgna-Pignatti C (2007) Surviving with thalassaemia major: the Italian experience. Pediatr Hematol Oncol 24:75–78

    Article  PubMed  Google Scholar 

  19. Borgna-Pignatti C, Vergine G, Lombardo T, Cappellini MD, Cianciulli P, Maggio A, Renda D, Lai ME, Mandas A, Forni G, Piga A, Bisconte MG (2004) Hepatocellular carcinoma in the thalassaemia syndromes. Br J Haematol 124(1):114–117

    Article  PubMed  Google Scholar 

  20. Lovet JM, Burroughs A, Bruix J (2003) Hepatocellural carcinoma. Lancet 362:1907–1917

    Article  Google Scholar 

  21. Mancuso A, Sciarrino E, Renda MC, Maggio A (2006) A prospective study of hepatocellular carcinoma incidence in thalassaemia. Hemoglobin 30(1):119–124

    Article  PubMed  CAS  Google Scholar 

Download references

Acknowledgments

The authors would like to thank the patients, their families and the medical and nursing staff of the following centers: General Hospital of Athens "Laiko", Children’s University Hospital of Athens "Agia Sofia", University General Hospital of Thessaloniki AHEPA, University Hospital of Thessaloniki Ippokrateion, University Hospital of Patras, University Hospital Hippocration of Athens, University Hospital of Larissa, General Hospital "Achilopouleio" of Volos, "DRAKOPOULEIO" Blood Centre of Athens, University Hospital of Ioannina, Venizeleio Hospital of Heraklion, General Hospital of Corfu, General Hospital of Karditsa, General Hospital of Corinthos, "Karamandaneio" Children's Hospital, Children’s Hospital of Penteli, General Hospital of Rhodes, Children's Hospital "Pan. & Aglaia Kyriakou" of Athens, General Hospital "Agios Panteleimon" of Nikea, General Hospital of Chalkida, General Hospital "Agios Pavlos" of Thessaloniki, General Hospital of Chania, General Hospital of Kalamata, General Hospital Vostanion of Mytilini, General Hospital "G. Gennimatas" Athens, General Hospital of Xanthi, General Hospital of Trikala, General Hospital of Arta, General Hospital of Serres, Social Security Institute of Pireaus, General Hospital of Sparte, University Hospital of Alexandroupoli, University Hospital of Heraklio "PEPAGNI", General Hospital of Katerini, General Hospital of Kavala, General Hospital of Kozani "Mamatseio", Hospital "Chatzikostas" of Ioannina, General Hospital of Amaliada, General Hospital of Rethymno, General Hospital of Kos, General Hospital of Athens “Aretaieio”, General Hospital of Tripoli, Children’s Hospital of Athens "Agia Sofia". Furthermore, the authors want to especially thank Mrs. Maria Tsalkani for her effort to collect this large amount of data in a limited period of time.

Author information

Authors and Affiliations

  1. Thalassaemia Center, Laikon General Hospital, 16 Sevastoupoleos street, GR-11526, Athens, Greece

    Ersi Voskaridou

  2. Children’s University Hospital of Athens ‘‘Agia Sofia’’, Athens, Greece

    Vasilis Ladis & Antonis Kattamis

  3. University General Hospital of Thessaloniki AHEPA, Thessaloniki, Greece

    Eleni Hassapopoulou

  4. University Hospital of Thessaloniki Ippokrateion, Thessaloniki, Greece

    Marina Economou

  5. University Hospital of Patras, Rio, Greece

    Alexandra Kourakli

  6. University Hospital Hippocration of Athens, Athens, Greece

    Konstantinos Maragkos

  7. University Hospital of Larissa, Larissa, Greece

    Kalliopi Kontogianni

  8. General Hospital ‘‘Achilopouleio’’ of Volos, Volos, Greece

    Stilianos Lafioniatis

  9. ‘‘DRAKOPOULEIO’’ Blood Centre of Athens, Athens, Greece

    Eleni Vrettou

  10. University Hospital of Ioannina, Ioannina, Greece

    Freideriki Koutsouka

  11. Venizeleio Hospital of Heraklion, Heraklion, Greece

    Alexandros Papadakis

  12. General Hospital of Corfu, Corfu, Greece

    Andreas Mihos

  13. General Hospital of Karditsa, Karditsa, Greece

    Eftihios Eftihiadis

  14. General Hospital of Corinthos, Corinth, Greece

    Kallistheni Farmaki

  15. ‘‘Karamandaneio’’ Children’s Hospital, Patras, Greece

    Ourania Papageorgiou

  16. Children’s Hospital of Penteli, Athens, Greece

    Georgia Tapaki

  17. General Hospital of Rhodes, Rhodes, Greece

    Polixeni Maili

  18. Children’s Hospital ‘‘Pan. and Aglaia Kyriakou’’ of Athens, Athens, Greece

    Maria Theohari

  19. General Hospital ‘‘Agios Panteleimon’’ of Nikea, Piraeus, Greece

    Marouso Drosou

  20. General Hospital of Chalkida, Chalkida, Greece

    Zafeiris Kartasis

  21. General Hospital ‘‘Agios Pavlos’’ of Thessaloniki, Thessaloniki, Greece

    Maria Aggelaki

  22. General Hospital of Chania, Chania, Greece

    Artemis Basileiadi

  23. General Hospital of Kalamata, Kalamata, Greece

    Ioannis Adamopoulos

  24. General Hospital Vostanion of Mytilini, Mytilini, Greece

    Ioannis Lafiatis

  25. General Hospital ‘‘G. Gennimatas’’ Athens, Athens, Greece

    Athanasios Galanopoulos

  26. General Hospital of Xanthi, Xanthi, Greece

    Georgios Xanthopoulidis

  27. General Hospital of Trikala, Trikala, Greece

    Efthimia Dimitriadou

  28. General Hospital of Arta, Arta, Greece

    Agapi Mprimi

  29. General Hospital of Serres, Serres, Greece

    Maria Stamatopoulou

  30. Social Security Institute of Pireaus, Pireaus, Greece

    Elanso Damba Haile

  31. General Hospital of Sparta, Sparta, Greece

    Maria Tsironi

  32. University Hospital of Alexandroupoli, Alexandroupoli, Greece

    Athanasios Anastasiadis

  33. University Hospital of Heraklio ‘‘PEPAGNI’’, Heraklion, Greece

    Maria Kalmanti

  34. General Hospital of Katerini, Katerini, Greece

    Margarita Papadopoulou

  35. General Hospital of Kavala, Kavala, Greece

    Evaggelia Panori

  36. General Hospital of Kozani ‘‘Mamatseio’’, Kozani, Greece

    Peristera Dimoxenou

  37. Hospital ‘‘Chatzikostas’’ of Ioannina, Ioannina, Greece

    Antigoni Tsirka

  38. General Hospital of Amaliada, Amaliada, Greece

    Dimitrios Georgakopoulos

  39. General Hospital of Rethymno, Rethymno, Greece

    Pantelis Drandrakis

  40. General Hospital of Kos, Kos, Greece

    Dionisia Dionisopoulou

  41. General Hospital of Athens ‘‘Aretaieio’’, Athens, Greece

    Androniki Ntalamaga

  42. General Hospital of Tripoli, Tripoli, Greece

    Ioannis Davros

  43. Children’s Hospital of Athens ‘‘Agia Sofia’’, Athens, Greece

    Markisia Karagiorga

Authors
  1. Ersi Voskaridou
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Vasilis Ladis
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Antonis Kattamis
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Eleni Hassapopoulou
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Marina Economou
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Alexandra Kourakli
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Konstantinos Maragkos
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Kalliopi Kontogianni
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Stilianos Lafioniatis
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Eleni Vrettou
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Freideriki Koutsouka
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Alexandros Papadakis
    View author publications

    You can also search for this author in PubMed Google Scholar

  13. Andreas Mihos
    View author publications

    You can also search for this author in PubMed Google Scholar

  14. Eftihios Eftihiadis
    View author publications

    You can also search for this author in PubMed Google Scholar

  15. Kallistheni Farmaki
    View author publications

    You can also search for this author in PubMed Google Scholar

  16. Ourania Papageorgiou
    View author publications

    You can also search for this author in PubMed Google Scholar

  17. Georgia Tapaki
    View author publications

    You can also search for this author in PubMed Google Scholar

  18. Polixeni Maili
    View author publications

    You can also search for this author in PubMed Google Scholar

  19. Maria Theohari
    View author publications

    You can also search for this author in PubMed Google Scholar

  20. Marouso Drosou
    View author publications

    You can also search for this author in PubMed Google Scholar

  21. Zafeiris Kartasis
    View author publications

    You can also search for this author in PubMed Google Scholar

  22. Maria Aggelaki
    View author publications

    You can also search for this author in PubMed Google Scholar

  23. Artemis Basileiadi
    View author publications

    You can also search for this author in PubMed Google Scholar

  24. Ioannis Adamopoulos
    View author publications

    You can also search for this author in PubMed Google Scholar

  25. Ioannis Lafiatis
    View author publications

    You can also search for this author in PubMed Google Scholar

  26. Athanasios Galanopoulos
    View author publications

    You can also search for this author in PubMed Google Scholar

  27. Georgios Xanthopoulidis
    View author publications

    You can also search for this author in PubMed Google Scholar

  28. Efthimia Dimitriadou
    View author publications

    You can also search for this author in PubMed Google Scholar

  29. Agapi Mprimi
    View author publications

    You can also search for this author in PubMed Google Scholar

  30. Maria Stamatopoulou
    View author publications

    You can also search for this author in PubMed Google Scholar

  31. Elanso Damba Haile
    View author publications

    You can also search for this author in PubMed Google Scholar

  32. Maria Tsironi
    View author publications

    You can also search for this author in PubMed Google Scholar

  33. Athanasios Anastasiadis
    View author publications

    You can also search for this author in PubMed Google Scholar

  34. Maria Kalmanti
    View author publications

    You can also search for this author in PubMed Google Scholar

  35. Margarita Papadopoulou
    View author publications

    You can also search for this author in PubMed Google Scholar

  36. Evaggelia Panori
    View author publications

    You can also search for this author in PubMed Google Scholar

  37. Peristera Dimoxenou
    View author publications

    You can also search for this author in PubMed Google Scholar

  38. Antigoni Tsirka
    View author publications

    You can also search for this author in PubMed Google Scholar

  39. Dimitrios Georgakopoulos
    View author publications

    You can also search for this author in PubMed Google Scholar

  40. Pantelis Drandrakis
    View author publications

    You can also search for this author in PubMed Google Scholar

  41. Dionisia Dionisopoulou
    View author publications

    You can also search for this author in PubMed Google Scholar

  42. Androniki Ntalamaga
    View author publications

    You can also search for this author in PubMed Google Scholar

  43. Ioannis Davros
    View author publications

    You can also search for this author in PubMed Google Scholar

  44. Markisia Karagiorga
    View author publications

    You can also search for this author in PubMed Google Scholar

Consortia

on behalf of the Greek Haemoglobinopathies Study Group

Corresponding author

Correspondence to Ersi Voskaridou.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Voskaridou, E., Ladis, V., Kattamis, A. et al. A national registry of haemoglobinopathies in Greece: Deducted demographics, trends in mortality and affected births. Ann Hematol 91, 1451–1458 (2012). https://doi.org/10.1007/s00277-012-1465-7

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00277-012-1465-7

Keywords

Search

Navigation