Download PDF
Semin Thromb Hemost 2005; 31(5): 569-576
DOI: 10.1055/s-2005-922229
Copyright © 2005 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

Epidemiology of von Willebrand Disease in Developing Countries

Alok Srivastava1 , Francesco Rodeghiero2
  • 1Professor, Department of Haematology, Christian Medical College, Vellore, India
  • 2Department of Haematology, San Bortolo Hospital, Vicenza, Italy
Further Information

Publication History

Publication Date:
08 November 2005 (online)

    Permissions and Reprints

ABSTRACT

There is limited information on von Willebrand disease (vWD) in developing countries. A questionnaire survey in 1998 showed that overall, less than one third of the expected number of patients with vWD in the population had been detected. Among those registered, the proportion with the severe form of the disease was relatively higher, forming up to 50% of the total, particularly in those countries with high prevalence of consanguineous marriages. Diagnosis was based on von Willebrand factor antigen (vWF:Ag) assays, vWF ristocetin cofactor (vWF:Rco) activity, and ristocetin-induced platelet aggregation (RIPA) at the reporting centers, but vWF multimer analysis was not widely available. Responsiveness to desmopressin acetate (DDAVP), which could be very significant clinically, was not often assessed. An attempt was made to assess accuracy of reporting of the prevalence of vWD by conducting a second survey aimed at normalizing the reported numbers with that of severe hemophilia A in a defined population on the presumption that the latter is less likely to be missed. These data showed that in most countries, the ratio of patients with vWD to severe hemophilia varied between 0.1 to 0.6 with a mean of approximately 0.4, as opposed to an expected ratio of approximately 1 (based on population-based data from Italy), confirming the underdiagnosis of vWD even within the same health-care system. A system for collecting more extensive and detailed data on vWD from developing countries (where 80% of the world’s population lives) is required. Efforts are needed to develop national registries and make at least basic services for diagnosis and treatment widely available.

KEYWORDS

von Willebrand disease - epidemiology - prevalence - underdiagnosis

REFERENCES

  • 1 Sadler J E, Mannucci P M, Berntorp E et al.. Impact, diagnosis and treatment of von Willebrand disease.  Thromb Haemost. 2000;  84 160-174
    Article in Thieme ConnectPubMedGoogle Scholar
  • 2 Mannucci P M. Desmopressin (DDAVP) in the treatment of bleeding disorder in the first 20 years.  Blood. 1997;  90 2515-2521
    PubMedGoogle Scholar
  • 3 Rodeghiero F, Castman G, Dini E. Epidemiological investigations of the prevalence of von Willebrand’s disease.  Blood. 1987;  69 454-459
    PubMedGoogle Scholar
  • 4 Sadler J E. A revised classification of von Willebrand disease.  Thromb Haemost. 1994;  71 520-525
    Article in Thieme ConnectPubMedGoogle Scholar
  • 5 Mazurier C, Meyer D. Molecular basis of von Willebrand disease.  Baillieres Clin Haematol. 1996;  9 229-241
    PubMedGoogle Scholar
  • 6 The University of Sheffield ISTH SSC vWF data base. Available at: http://www.shef.ac.uk/vwf/index.html Accessed September 23, 2005
    Google Scholar
  • 7 Scott J P, Montgomery R R. Therapy of von Willebrand disease.  Semin Thromb Hemost. 1993;  19 37-47
    PubMedGoogle Scholar
  • 8 von Willebrand Working Party . Treatment and management of von Willebrand Disease.  Haemophilia. 1997;  3(suppl 2) 4-8
    PubMedGoogle Scholar
  • 9 Plaimauer B, Schlokat U, Turecek P L et al.. Recombinant von Willebrand factor: preclinical development.  Semin Thromb Hemost. 2001;  27 395-403
    Article in Thieme ConnectPubMedGoogle Scholar
  • 10 The World Development Report, 2002. Geneva; United Nations Development Program 2002
    Google Scholar
  • 11 Werner E J, Broxson E H, Tucker E L, Dgiroux D S, Shults J, Abshire T C. Prevalence of von Willebrand disease in children: a multiethnic study.  J Pediatr. 1993;  123 893-898
    CrossrefPubMedGoogle Scholar
  • 12 Shoa’i I, Lavergne J M, Ardaillou N, Obert B, Ala F, Meyer D. Heterogeneity of von Willebrand’s disease: study of 40 Iranian cases.  Br J Haematol. 1977;  37 67-83
    PubMedGoogle Scholar
  • 13 Bird A R, Shuttleworth M, Anderson C, Karabus C. von Willebrand disease in the Western Cape.  S Afr Med J. 1996;  86 261-263
    PubMedGoogle Scholar
  • 14 Cabrera M E, Artigas C G, Paez E et al.. von Willebrand’s disease in the IX region of Chile.  Rev Med Chil. 1989;  117 423-430
    PubMedGoogle Scholar
  • 15 Diez-Ewald M, Vizcaino G, Arteaga-Vizcaino M, Fernandez N, Weir-Medina J, Gomez O. Epidemiology of von Willebrand’s disease in the state of Zulia, Venezuela.  Invest Clin. 1991;  32 187-199
    PubMedGoogle Scholar
  • 16 Srivastava A, Chuansumrit A, Chandy M, Duraiswamy G D, Karabus C. Management of hemophilia in the developing world.  Haemophilia. 1998;  4 474-480
    CrossrefPubMedGoogle Scholar
  • 17 Zhang L, Li H, Zhao H, Zhang X, Ji L, Yang R. Retrospective analysis of 1312 patients with haemophilia and related disorders in a single Chinese institute.  Haemophilia. 2003;  9 696-702
    CrossrefPubMedGoogle Scholar
  • 18 Islam S I, Quadri M I. Spectrum of hereditary coagulation factor deficiencies in eastern province, Saudi Arabia.  East Mediterr Health J. 1999;  5 1188-1195
    PubMedGoogle Scholar
  • 19 Karimi M, Yarmohammadi H, Ardeshiri R, Yarmohammadi H. Inherited coagulation disorders in southern Iran.  Haemophilia. 2002;  8 740-744
    CrossrefPubMedGoogle Scholar
  • 20 Mannucci P M, Bloom A L, Larrieu M J, Nilsson I M, West R R. Atherosclerosis and von Willebrand factor 1. Prevalence of severe von Willebrand’s disease in Western Europe and Israel.  Br J Haematol. 1984;  57 163-169
    PubMedGoogle Scholar
  • 21 Mueller R F, Cook J. Mendelian inheritance. In: Rimoin DL, Connor JM, Pyeritz RE Principles and Practice of Medical Genetics. 3rd ed. New York; Churchill Livingstone 1997: 87-102
    Google Scholar
  • 22 Berliner S A, Seligsohn U, Zivelin A, Zwang E, Sofferman G. A relatively high frequency of severe (type III) von Willebrand’s disease in Israel.  Br J Haematol. 1986;  62 535-543
    PubMedGoogle Scholar
  • 23 Fischer R R, Lerner C, Bandinelli E, Fonseca A S, Roisenberg I. Inheritance and prevalence of von Willebrand’s disease severe form in a Brazilian population.  J Inherit Metab Dis. 1989;  12 293-301
    CrossrefPubMedGoogle Scholar
  • 24 Awidi A S. A study of von Willebrand’s disease in Jordon.  Ann Hematol. 1992;  64 299-302
    CrossrefPubMedGoogle Scholar
  • 25 Quiroga T, Perez M, Pereira J, Mezzano D. Diagnosis of von Willebrand’s disease subtypes by analysis of multimeric composition of plasma von Willebrand factor.  Rev Med Chil. 1993;  121 987-993
    PubMedGoogle Scholar
  • 26 Mahasandana C, Suvatte V, Pung-amrit P, Tuchinda S. von Willebrand’s disease: Studies of platelet functions, factor VIII procoagulant activity (F VIII C) and factor VIII-related antigen (F VIII Rag) in three families.  Southeast Asian J Trop Med Public Health. 1979;  10 243-247
    PubMedGoogle Scholar
  • 27 Mehta B C, Agarwal M B. Inherited coagulation disorders in India.  Indian J Pediatr. 1981;  48 525-531
    CrossrefPubMedGoogle Scholar
  • 28 Kulkarni M L, Nanda G. Hemophiliacs-a picture from a developing country: Karnataka, a south Indian state.  Southeast Asian J Trop Med Public Health. 1993;  24(suppl 1) 270-273
    PubMedGoogle Scholar
  • 29 A1-Fawaz I M, Gader A MA, Bahakim H M, Al-Momen A K, Harakati M S. Hereditary bleeding disorders in Riyadh, Saudi Arabia.  Ann Saudi Med. 1996;  16 257-261
    CrossrefPubMedGoogle Scholar
  • 30 Quiroga T, Perez M, Rodriguez S et al.. Skin and mucous membrane hemorrhages: clinical assessment, study sequence and relative frequency of hereditary diseases of hemostasis in a Chilean population.  Rev Med Chil. 1997;  125 409-418
    PubMedGoogle Scholar
  • 31 Mehta B C, Agarwal M B, Bhanotre P C, Rao S M. Inheritance pattern of von Willebrand’s disease-a study of 26 cases.  Indian Pediatr. 1982;  19 665-668
    PubMedGoogle Scholar
  • 32 Almaani W S, Awidi A S. Spontaneous intracranial haemorrhage secondary to von Willebrand’s disease.  Surg Neurol. 1986;  26 457-460
    PubMedGoogle Scholar
  • 33 Tsay W, Shen M C. Experience of desmopressin (DDAVP) administration in patients with congenital and acquired bleeding disorders.  J Formos Med Assoc. 1992;  91 962-969
    PubMedGoogle Scholar
  • 34 Chuansumrit A, Hathirat P, Pintadit P, Isarangkura P. Response of patients with bleeding disorder to DDAVP administration.  Southeast Asian J Trop Med Public Health. 1993;  24(suppl 1) 174-179
    PubMedGoogle Scholar
  • 35 Sivasankaran P, Prasad H S, Nalini P. von Willebrand’s disease in identical twins.  Indian J Pediatr. 1989;  56 435-437
    PubMedGoogle Scholar
  • 36 Grand B, Blanco A, Riveros D et al.. Congenital factor XIII deficiency associated with von Willebrand disease.  Am J Hematol. 1990;  35 208-209
    PubMedGoogle Scholar
  • 37 Paul B, Nkrumah F K, Bird A R, Kossew B P, Jacobs P. von Willebrand’s disease in Zimbabwe.  S Afr Med J. 1991;  80 153-155
    PubMedGoogle Scholar
  • 38 Dharnidharka V R, Bichile S K, Vaidya S S. von Willebrand’s disease with gastrointestinal telangiectasia.  J Assoc Physicians India. 1993;  41 395-396
    PubMedGoogle Scholar
  • 39 Ahuja R, Kripalani A, Choudhary V P, Takkar D. von Willebrand disease: a rare cause of puberty menorrhagia.  Aust N Z J Obstet Gynaecol. 1995;  35 337-338
    PubMedGoogle Scholar
  • 40 Nathwani A C, Tuddenham EGD. Epidemiology of coagulation disorders.  Baillieres Clin Haematol. 1992;  5 383-439
    PubMedGoogle Scholar
  • 41 World Federation of Haemophilia .Status and Atlas of Hemophilia Worldwide, 1984. Munich, Germany; World Federation of Haemophilia Clearing House 1984
    Google Scholar
  • 42 Anonymous. World Economic and Social Survey 1997. New York; United Nations 1997
    Google Scholar
  • 43 Scientific Subcommittee Reports .1999 Minutes and Annual Reports: Scientific Subcommittees of the Scientific and Standardization Committee International Society on Thrombosis and Haemostasis. http://www.med.unc.edu/isth/SSC/99minutes/toc99.htm. Accessed September 23, 2005
    Google Scholar
  • 44 Soucie J R, Evatt B, Jackson D. and the Hemophilia Surveillance System Project Investigators. . Occurrence of hemophilia in the United States.  Am J Hematol. 1998;  59 288-294
    CrossrefPubMedGoogle Scholar
  • 45 Anonymous. Report on the World Social Situation, 1997. New York; United Nations 1997
    Google Scholar
  • 46 Anonymous. Control of Haemophilia: Haemophilia Care in Developing Countries. Report of a joint WHO/World Federation of Haemophilia meeting. Geneva; World Health Organisation 1997
    Google Scholar
  • 47 Srivastava A. The delivery of haemophilia care in the developing world.  Haemophilia. 1998;  4(suppl 2) 33-40
    PubMedGoogle Scholar

Alok SrivastavaM.D. 

Department of Haematology, Christian Medical College Vellore-632004, India

Email: aloks@cmcvellore.ac.in