Spectrum of inherited bleeding disorders from Western India

M. Manisha, K. Ghosh, S. Shetty, S. Nair, A. Khare, B. Kulkarni, A. V. Pathare, S. Baindur, D. Mohanty

Research output: Contribution to journalArticle

12 Citations (Scopus)

Abstract

Objective: The study was undertaken to assess the magnitude and diversity of different bleeding disorders in Western India. Materials and methods: 768 cases referred to our Institute for evaluation of an underlying bleeding diathesis were investigated appropriately to detect the cause of the abnormal hemostatic function. Results: 630 patients were diagnosed to have hereditary bleeding diathesis. Amongst these, 598 patients had a coagulation disorder while only 32 patients had a platelet function abnormality. Amongst the coagulation disorders, hemophilia A (70.5%) was the most common disorder followed by hemophilia B (14%) and VWD (10.8%). Glanzman's thrombasthenia (84.3%) was the most common platelet function disorder followed by Bernard-Soulier syndrome (12.5%). Some rare disorders have also been diagnosed. Conclusion: In spite of their apparent rarity, India has a substantial number of cases of inherited bleeding disorders. A large number of these patients is referred to many tertiary care institutions. It is therefore desirable that district hospitals must develop their laboratories to detect most of these disorders so that the patients need not travel long distances to get an appropriate diagnosis and proper management. All 1st degree female relatives of severe and moderate hemophilia must get factor assays done because some of them may be vulnerable to post-procedural or post-traumatic bleeding.

Original languageEnglish
Pages (from-to)39-47
Number of pages9
JournalHaematologia
Volume32
Issue number1
DOIs
Publication statusPublished - 2002

Fingerprint

India
Hemorrhage
Disease Susceptibility
Hemophilia A
Blood Platelets
Bernard-Soulier Syndrome
Thrombasthenia
Hemophilia B
District Hospitals
Hemostatics
Tertiary Healthcare

Keywords

  • Bleeding diathesis
  • Congenital
  • Hemophilia
  • Inherited

ASJC Scopus subject areas

  • Hematology

Cite this

Manisha, M., Ghosh, K., Shetty, S., Nair, S., Khare, A., Kulkarni, B., ... Mohanty, D. (2002). Spectrum of inherited bleeding disorders from Western India. Haematologia, 32(1), 39-47. https://doi.org/10.1163/156855902760262754

Spectrum of inherited bleeding disorders from Western India. / Manisha, M.; Ghosh, K.; Shetty, S.; Nair, S.; Khare, A.; Kulkarni, B.; Pathare, A. V.; Baindur, S.; Mohanty, D.

In: Haematologia, Vol. 32, No. 1, 2002, p. 39-47.

Research output: Contribution to journalArticle

Manisha, M, Ghosh, K, Shetty, S, Nair, S, Khare, A, Kulkarni, B, Pathare, AV, Baindur, S & Mohanty, D 2002, 'Spectrum of inherited bleeding disorders from Western India', Haematologia, vol. 32, no. 1, pp. 39-47. https://doi.org/10.1163/156855902760262754
Manisha M, Ghosh K, Shetty S, Nair S, Khare A, Kulkarni B et al. Spectrum of inherited bleeding disorders from Western India. Haematologia. 2002;32(1):39-47. https://doi.org/10.1163/156855902760262754
Manisha, M. ; Ghosh, K. ; Shetty, S. ; Nair, S. ; Khare, A. ; Kulkarni, B. ; Pathare, A. V. ; Baindur, S. ; Mohanty, D. / Spectrum of inherited bleeding disorders from Western India. In: Haematologia. 2002 ; Vol. 32, No. 1. pp. 39-47.
@article{1b05c2849a2540e39eb3447097e57725,
title = "Spectrum of inherited bleeding disorders from Western India",
abstract = "Objective: The study was undertaken to assess the magnitude and diversity of different bleeding disorders in Western India. Materials and methods: 768 cases referred to our Institute for evaluation of an underlying bleeding diathesis were investigated appropriately to detect the cause of the abnormal hemostatic function. Results: 630 patients were diagnosed to have hereditary bleeding diathesis. Amongst these, 598 patients had a coagulation disorder while only 32 patients had a platelet function abnormality. Amongst the coagulation disorders, hemophilia A (70.5{\%}) was the most common disorder followed by hemophilia B (14{\%}) and VWD (10.8{\%}). Glanzman's thrombasthenia (84.3{\%}) was the most common platelet function disorder followed by Bernard-Soulier syndrome (12.5{\%}). Some rare disorders have also been diagnosed. Conclusion: In spite of their apparent rarity, India has a substantial number of cases of inherited bleeding disorders. A large number of these patients is referred to many tertiary care institutions. It is therefore desirable that district hospitals must develop their laboratories to detect most of these disorders so that the patients need not travel long distances to get an appropriate diagnosis and proper management. All 1st degree female relatives of severe and moderate hemophilia must get factor assays done because some of them may be vulnerable to post-procedural or post-traumatic bleeding.",
keywords = "Bleeding diathesis, Congenital, Hemophilia, Inherited",
author = "M. Manisha and K. Ghosh and S. Shetty and S. Nair and A. Khare and B. Kulkarni and Pathare, {A. V.} and S. Baindur and D. Mohanty",
year = "2002",
doi = "10.1163/156855902760262754",
language = "English",
volume = "32",
pages = "39--47",
journal = "Haematologia",
issn = "0017-6559",
publisher = "VSP BV",
number = "1",

}

TY - JOUR

T1 - Spectrum of inherited bleeding disorders from Western India

AU - Manisha, M.

AU - Ghosh, K.

AU - Shetty, S.

AU - Nair, S.

AU - Khare, A.

AU - Kulkarni, B.

AU - Pathare, A. V.

AU - Baindur, S.

AU - Mohanty, D.

PY - 2002

Y1 - 2002

N2 - Objective: The study was undertaken to assess the magnitude and diversity of different bleeding disorders in Western India. Materials and methods: 768 cases referred to our Institute for evaluation of an underlying bleeding diathesis were investigated appropriately to detect the cause of the abnormal hemostatic function. Results: 630 patients were diagnosed to have hereditary bleeding diathesis. Amongst these, 598 patients had a coagulation disorder while only 32 patients had a platelet function abnormality. Amongst the coagulation disorders, hemophilia A (70.5%) was the most common disorder followed by hemophilia B (14%) and VWD (10.8%). Glanzman's thrombasthenia (84.3%) was the most common platelet function disorder followed by Bernard-Soulier syndrome (12.5%). Some rare disorders have also been diagnosed. Conclusion: In spite of their apparent rarity, India has a substantial number of cases of inherited bleeding disorders. A large number of these patients is referred to many tertiary care institutions. It is therefore desirable that district hospitals must develop their laboratories to detect most of these disorders so that the patients need not travel long distances to get an appropriate diagnosis and proper management. All 1st degree female relatives of severe and moderate hemophilia must get factor assays done because some of them may be vulnerable to post-procedural or post-traumatic bleeding.

AB - Objective: The study was undertaken to assess the magnitude and diversity of different bleeding disorders in Western India. Materials and methods: 768 cases referred to our Institute for evaluation of an underlying bleeding diathesis were investigated appropriately to detect the cause of the abnormal hemostatic function. Results: 630 patients were diagnosed to have hereditary bleeding diathesis. Amongst these, 598 patients had a coagulation disorder while only 32 patients had a platelet function abnormality. Amongst the coagulation disorders, hemophilia A (70.5%) was the most common disorder followed by hemophilia B (14%) and VWD (10.8%). Glanzman's thrombasthenia (84.3%) was the most common platelet function disorder followed by Bernard-Soulier syndrome (12.5%). Some rare disorders have also been diagnosed. Conclusion: In spite of their apparent rarity, India has a substantial number of cases of inherited bleeding disorders. A large number of these patients is referred to many tertiary care institutions. It is therefore desirable that district hospitals must develop their laboratories to detect most of these disorders so that the patients need not travel long distances to get an appropriate diagnosis and proper management. All 1st degree female relatives of severe and moderate hemophilia must get factor assays done because some of them may be vulnerable to post-procedural or post-traumatic bleeding.

KW - Bleeding diathesis

KW - Congenital

KW - Hemophilia

KW - Inherited

UR - http://www.scopus.com/inward/record.url?scp=0036901954&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0036901954&partnerID=8YFLogxK

U2 - 10.1163/156855902760262754

DO - 10.1163/156855902760262754

M3 - Article

VL - 32

SP - 39

EP - 47

JO - Haematologia

JF - Haematologia

SN - 0017-6559

IS - 1

ER -