Epidemiology of Haemophilia in Greece: An Overview

 

PDF Download Buy Article Permissions and Reprints

Summary

Demographic data of the Greek haemophilia A and B population for the period 1972-1993 were analyzed. Prevalence at birth including known not-registered patients was calculated at 23.1 per 100,000 male births. However, the observed prevalence in 1993 was only 61% of the expected. Since 1975 the proportion of mild cases had significantly increased. Adjusted by age, severity and HIV status reproductive fitness of haemophiliacs was 0.62. Overall mortality was 2.6 times higher than in the general population, but 7.9 times among patients with severe haemophilia and 16.4 among HIV(+) haemophiliacs. Fifty out of 78 deaths occurred among HIV(+) patients and 28 of these were caused by AIDS. Inhibitor patients did not show excess mortality due to bleeding. Cancer mortality was equal to normal, but the number of deaths from ischaemic heart disease was 0.25 of the expected. Risk of death due to cerebral haemorrhage was 3.8 times higher in HIV(+) haemophiliacs than in HIV(-).

• References

• 1 Mandalaki T. General principles of haemophilia care; retrospective review and perspectives. In: Hemophilia and von Willebrand’s disease in the 1990’s Lusher JM, Kessler CM. eds. Amsterdam: Elsevier Science Publishers; B.V.: 1991. pp 3-8
• 2 Rosendaal FR, Briët E. The increasing prevalence of haemophilia. Thromb Haemost 1989; 63: 145
  PubMedGoogle Scholar
• 3 National Service of Statistics. Statistical Yearbooks, Athens 1953-1991
  PubMedGoogle Scholar
• 4 Mandalaki T, Koumbarelis E, Gialeraki A, Apostolou A. Family studies in an extremely large mild Haemophilia A pedigree including 10% of the Greek haemophiliacs. Br J Haemotol 1994 in press
  PubMedGoogle Scholar
• 5 Larsson SA. Life expectancy of Swedish Haemophiliacs, 1831–1980. Br J Haematol 1985; 59: 593-602
  CrossrefPubMedGoogle Scholar
• 6 Aronson DL. Cause of death in hemophilia A patients in the United States from 1968 to 1979. Am J Hematol 1988; 27: 7-12
  CrossrefPubMedGoogle Scholar
• 7 Erfle V, Heihmann R, Melbert W, Krüger G, Seifried E, Heimpel H, Rasokat H, Lechler E, Holzer E, Hellstern P, Köhler M, Goebel FD. Prevalence of antibodies to HTLV-III in AIDS risk groups in West Germany. Cancer Res 1985; 45 Suppl 4627s-4629s
  PubMedGoogle Scholar
• 8 Allain JP. Prevalence of HTLV-III/LAV antibodies in patients with hemophilia and in their sexual partners in France. N Engl J Med 1986; 315: 517-518
  CrossrefPubMedGoogle Scholar
• 9 AIDS group for the United Kingdom Haemophilia Centre Directors: Prevalence of antibody to HTLV-III in haemophiliacs in the United Kingdom. Br Med J 1986 293. 175-176
  PubMedGoogle Scholar
• 10 Rosendaal FR, Smit C, Varekamp I, Bröcker-Vriends A, Suurmeijer TPBM, Briët E. AIDS and haemophilia: a study among Dutch haemophiliacs on the psychological impact of the AIDS threat, the prevalence of HIV antibodies and the adoption of measures to prevent HIV transmission. Haemostasis 1988; 18: 73-82
  PubMedGoogle Scholar
• 11 Rouzioux C, Brun-Venizet F, Couroucé AM, Gazengel C, Vergoz D, Desmyter J, Vermylen J, Vermylen C, Klatzmann D, Geroldi D, Barreau C, Barreé-Sinoussi F, Chermann JC, Christol D, Montagnier L. Immunoglobulin G antibodies to lymphadenopathy associated virus in differently treated French and Belgian hemophiliacs. Ann Int Med 1985; 102: 476-479
  CrossrefPubMedGoogle Scholar
• 12 Mandalaki T. Management of haemophilia in Greece. Thromb Haemost 1976; 35: 522-530
  Article in Thieme ConnectPubMedGoogle Scholar
• 13 Larsson SA, Nilsson IM, Blomback M. Current status of Swedish hemophiliacs. Acta Med Scand 1982; 212: 195-200
  PubMedGoogle Scholar
• 14 Markova I, Forbes CD, Inwood M. The consumers’ view of genetic counseling of hemophilia. Am J Med Genet 1984; 17: 741-752
  CrossrefPubMedGoogle Scholar
• 15 Haldane JBS. The mutation rate of the gene for hemophilia and its segregation rates in males and females. Ann Eugen (Lond) 1947; 13: 262-271
  PubMedGoogle Scholar
• 16 Francis RB, Kasper CK. Reproduction in hemophilia. JAMA 1983; 250: 3192-3195
  CrossrefPubMedGoogle Scholar
• 17 Istvan L, Czeizel A, Kerenyi M, Toth AM, Domby E. Genetic-Epidemiologic study of haemophilia A and B in Hungary. Hum Hered 1990; 40: 29-33
  CrossrefPubMedGoogle Scholar
• 18 Rosendaal FR, Smit C, Varekamp I, Bröcker-Vriends AHJT, Van Dijck H, Suurmeijer TPBM, Vandenbroucke JP, Briët E. Modem haemophilia treatment: medical improvements and quality of life. J Intern Med 1990; 228: 633-640
  CrossrefPubMedGoogle Scholar
• 19 Yannitsiotis A, Bossinakou I, Louizou C, Panayotopoulou C, Mandalaki T. Jaundice and hepatitis B surface antigen and antibody in Greek haemophiliacs. Scand J Haematol 1977; 30: 11-15
  PubMedGoogle Scholar
• 20 Rizza CR, Spooner RJD. Treatment of haemophilia and related disorders in Britain and Northern Ireland during 1976-1980: report on behalf of the directors of haemophilia centres in the United Kingdom. Br Med J 1983; 286: 929-933
  CrossrefPubMedGoogle Scholar
• 21 Hatzakis A, Hadziyannis S, Maclure M, Louizou C, Yannitsiotis A, Mandalaki T. Concurrent hepatitis B, delta and human immunodeficiency virus infection in hemophiliacs. Thromb Haemost 1987; 58: 791
  Article in Thieme ConnectPubMedGoogle Scholar
• 22 Ikkala E, Helske T, Myllylä G, Nevanlinna HR, Pitkanen P, Rasi V. Changes in life expectancy of patients with severe haemophilia A in Finland in 1930-79. Br J Haematol 1982; 52: 7-12
  CrossrefPubMedGoogle Scholar
• 23 Rosendaal FR, Varekamp I, Smit C, Bröcker-Vriends AHJT, Van Dijck H, Vandenbroucke JP, Hermans J, Suurmeijer TPBM, Briët E. Mortality and causes of death in Dutch haemophiliacs, 1973-1986. Br J Haematol 1989; 71: 71-76
  CrossrefPubMedGoogle Scholar
• 24 Rosendaal FR, Briët E, Stibbe J, van Herpen G, Gevers Leuven JA, Hofman A, Vandenbroucke JP. Hemophilia protects against ischemic heart disease: a study of risk factors. Br J Haematol 1990; 75: 525-530
  CrossrefPubMedGoogle Scholar