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

Downloads

Download data is not yet available.
Abstract
295
PDF
320

Downloads

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

Section

Original Papers