Pattern of Common Inherited Coagulation Disorders: Evaluation of 100 Cases

Authors

  • Mohammed Nuruzzaman Bhuiyan Classified Specialist in Pathology, AFIP, Dhaka, Bangladesh
  • Maj Gen Susane Giti Commandant, AFIP, Dhaka, Bangladesh
  • Mohammed Mosleh Uddin Classified Specialist in Pathology, BMT Centre, CMH, Dhaka, Bangladesh
  • Monwar Tarek Classified Specialist in Pathology, AFIP, Dhaka, Bangladesh
  • Lutfunnahar Khan Classified Specialist in Pathology, AFIP, Dhaka, Bangladesh

DOI:

https://doi.org/10.3329/jafmc.v15i1.48646

Keywords:

Hereditary coagulation defect, Von Willebrand disease, Rare inherited coagulation disorder

Abstract

Introduction: The bleeding disorders having inherited abnormality of haemostasis may present with significant difficulties in diagnosis and management. The overall frequency of these disorders in the general population is low.

Objectives: To study the frequency of inherited coagulation disorders and their prevalent clinical manifestations.

Materials and Methods: A prospective cross sectional study of one hundred patients of all age group and both sexes was organized as both in-patient and out-patient based in the Department of haematology, Armed Forces Institute of Pathology (AFIP) from July 2012 to June 2013.

Results: In this study, out of 100 patients, haemophilia A, diagnosed in 75% patients, was the most common disorder. Age group ranging from 5-15 years constituted 48% of total patients. Male to female ratio of patients having hereditary coagulation defect was 73:2 in haemophilia A whereas 2:3 in vWD. Consanguinity was documented in 60% of vWD and 24% of haemophilia A. The most common clinical symptom in hereditary coagulation defect (HCD) was echymosis (60%). The most common presenting feature of haemophilia was haematoma (57.95%) whereas menorrhagia was the most common presentation of female patients with vWD. Among 97 patients of haemophilia and vWD, 59(60.82%) cases were in mild form and 04(4.12%) cases were found to have severe coagulation defect. Fresh frozen plasma (FFP) is the most common modality of treatment in HCD.

Conclusion: It has become the need of the time to find out such a cost effective diagnostic parameter to make an early diagnosis of inherited coagulation disorders.

Journal of Armed Forces Medical College Bangladesh Vol.15 (1) 2019: 64-66

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Published

2020-08-20

How to Cite

Bhuiyan, M. N., Giti, M. G. S., Uddin, M. M., Tarek, M., & Khan, L. (2020). Pattern of Common Inherited Coagulation Disorders: Evaluation of 100 Cases. Journal of Armed Forces Medical College, Bangladesh, 15(1), 64–66. https://doi.org/10.3329/jafmc.v15i1.48646

Issue

Vol. 15 No. 1 (2019)

Section

Original Papers

License

Submission of a manuscript for publication implies the transfer of the copyright from the author to the publisher upon acceptance. Accepted manuscripts become the permanent property of the Journal of Armed Forces Medical College and may not be reproduced by any means in whole or in part without the written consent of the publisher.