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CC BY-NC-ND 4.0 · J Lab Physicians 2022; 14(04): 465-470
DOI: 10.1055/s-0042-1748827
Original Article

Spectrum of Inherited Bleeding Disorder with Special Reference to von Willebrand Disease in Eastern India

Nehal Ahmad
1   Department of Pathology, Hamdard Institute of Medical Sciences and Research, Jamia Hamdard, New Delhi, India
,
Subhajit Das
2   Divisional Medical Officer, Indian Railway, New Jalpaiguri, West Bengal, India
,
Jyoti Shukla
3   Department of Pathology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India
,
Mohammad Jaseem Hassan
4   Department of Pathology, J.N. Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India
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Abstract

Background The objective of this study is to study the prevalence, clinical spectrum, and hematological profile of inherited bleeding disorder with special reference to von Willebrand disease in eastern India.

Materials and Methods This prospective study was done in a tertiary care center in the eastern part of India over 2 years. Detailed history and clinical findings were noted in a proforma. Laboratory analysis included prothrombin time, activated partial thromboplastin time, bleeding time, and fibrinogen assay along with tests related to specific factor assay.

Results One hundred and five patients were diagnosed as suffering with the inherited bleeding disorder out of a total of 1,204 patients. The age of patients ranged from 13 days to 35 years. The most common presenting clinical feature was prolonged bleeding after cut (76.19%). Out of 105 patients, 97 patients (92.38%) had coagulation defect, 5 patients (4.76%) had von Willebrand disease (vWD), and 3 patients (2.85%) had platelet defect. Most common coagulation defect was hemophilia A (84 cases), followed by hemophilia B (8 cases). Other rare congenital factor deficiencies were seen in five cases (5.15%). Only platelet defect was Glanzmann's thrombasthenia (GT). The age of vWD patients ranged from 4.5 years to 24 years. Forty percent patients with vWD disease were type 1 followed by 40% of type 2N and 20% of type 3 vWD.

Conclusion vWD was not so common in eastern India. vWD was present only in 4.76% cases in this study. The most common coagulation defect was hemophilia A (86.59%) in our study. GT was present in only 2.85% cases.

Keywords

bleeding - Glanzmann's thrombasthenia - hemophilia - von Willebrand disease


Publication History

Article published online:
07 June 2022

© 2022. The Indian Association of Laboratory Physicians. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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  • References

  • 1 Baklaja R, Pesic MC, Czarnecki J. Inherited blood clotting disorders. In: Hemostasis and Hemorrhagic Disorders. 2nd ed. Thymus Mediz: Fachbuchver; 2008: 68-115
    Google Scholar
  • 2 Stonebraker JS, Bolton-Maggs PH, Soucie JM, Walker I, Brooker M. A study of variations in the reported haemophilia A prevalence around the world. Haemophilia 2010; 16 (01) 20-32
    CrossrefPubMedGoogle Scholar
  • 3 Mahlangu JN, Gilham A. Medical and Scientific Advisory Council of the South African Haemophilia Foundation. Guideline for the treatment of haemophilia in South Africa. S Afr Med J 2008; 98 (2 Pt 2): 126-140
    PubMedGoogle Scholar
  • 4 Laffan M, Brown SA, Collins PW. et al. The diagnosis of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors' Organization. Haemophilia 2004; 10 (03) 199-217
    CrossrefPubMedGoogle Scholar
  • 5 Srivastava A, Brewer AK, Mauser-Bunschoten EP. et al; Treatment Guidelines Working Group on Behalf of The World Federation Of Hemophilia. Guidelines for the management of hemophilia. Haemophilia 2013; 19 (01) e1-e47
    CrossrefPubMedGoogle Scholar
  • 6 Cattaneo M. Inherited platelet-based bleeding disorders. J Thromb Haemost 2003; 1 (07) 1628-1636
    CrossrefPubMedGoogle Scholar
  • 7 Gupta PK, Charan VD, Saxena R. Spectrum of Von Willebrand disease and inherited platelet function disorders amongst Indian bleeders. Ann Hematol 2007; 86 (06) 403-407
    CrossrefPubMedGoogle Scholar
  • 8 Sajid R, Khalid S, Mazari N, Azhar WB, Khurshid M. Clinical audit of inherited bleeding disorders in a developing country. Indian J Pathol Microbiol 2010; 53 (01) 50-53
    CrossrefPubMedGoogle Scholar
  • 9 Ahmad F, Kannan M, Ranjan R, Bajaj J, Choudhary VP, Saxena R. Inherited platelet function disorders versus other inherited bleeding disorders: an Indian overview. Thromb Res 2008; 121 (06) 835-841
    CrossrefPubMedGoogle Scholar
  • 10 Manisha M, Ghosh K, Shetty S. et al. Spectrum of inherited bleeding disorders from Western India. Haematologia (Budap) 2002; 32 (01) 39-47
    CrossrefPubMedGoogle Scholar
  • 11 Trasi S, Shetty S, Ghosh K, Mohanty D. Prevalence and spectrum of von Willebrand disease from western India. Indian J Med Res 2005; 121 (05) 653-658
    PubMedGoogle Scholar
  • 12 Kumar S, Kishore R, Gupta V, Jain M, Shukla J. Prevalence and spectrum of von Willebrand disease in Eastern Uttar Pradesh. Indian J Pathol Microbiol 2010; 53 (03) 486-489
    PubMedGoogle Scholar
  • 13 Borhany M, Shamsi T, Naz A. et al. Congenital bleeding disorders in Karachi, Pakistan. Clin Appl Thromb Hemost 2011; 17 (06) E131-E137
    CrossrefPubMedGoogle Scholar
  • 14 Scheibel E. Von Willebrand disease in Denmark: demography and treatment. Haemophilia 1999; 5 (Suppl. 02) 71
    CrossrefPubMedGoogle Scholar
  • 15 Awidi AS. A study of von Willebrand's disease in Jordan. Ann Hematol 1992; 64 (06) 299-302
    CrossrefPubMedGoogle Scholar
  • 16 Diez-Ewald M, Vizcaíno G, Arteaga-Vizcaíno M, Fernández N, Weir-Medina J, Gómez O. [Epidemiology of von Willebrand's disease in the state of Zulia, Venezuela]. Invest Clin 1991; 32 (04) 187-199
    PubMedGoogle Scholar
  • 17 Chuamary NG, Santosa L. Prevalence and clinico-hematologic scenario of von Willebrand disease in a population of Filipinos with bleeding tendency. Sysmex J Int 2006; 16 (02) 42-46
    Google Scholar
  • 18 Gupta PK, Ahmed RPH, Sazawal S, Choudhry VP, Saxena R. Relatively high frequency of VWD types 3 and 2 in a cohort of Indian patients: the role of multimeric analysis. J Thromb Haemost 2005; 16 (03) 1321-1322
    CrossrefPubMedGoogle Scholar