Epidemiological Profile of Patients with Hemophilia in Northern Pakistan: A Single-Center Study

Usama Azam; Tahira Zafar; Lubna Zafar; ASMA NAFISA; Sundas Durrani; Mehwish Iqbal; Nadeem Ikram

Authors

Usama Azam Research Officer, Hemophilia Patients Welfare Society Rawalpindi
Tahira Zafar Director, Hemophilia Treatment Center Rawalpindi
Lubna Zafar Professor of Pathology, Consultant Hematologist, FUMC/FFH
ASMA NAFISA Rawalpindi Medical University
Sundas Durrani Rawalpindi Medical University
Mehwish Iqbal Assisstant Prof . Rawalpindi Medical University
Nadeem Ikram Professor of Pathology

Keywords:

Hemophilia, bleeding, congenital, x-chromosome, knee joint.

Abstract

ABSTRACT

Introduction: Hemophilia is an inborn X-linked coagulation system disorder in which the affected individual is predisposed to bleeding for the remainder of his life due to a factor VIII (Hemophilia A) or factor IX (Hemophilia B) deficiency. Early and proper factor replacement therapy can prevent death from lethal bleeding; however, degrees of awareness vary. The level of understanding among students and general practitioners has remained low, resulting in treatment delays and ineffectiveness. The current study will focus on the symptoms, complications and care of children with Hemophilia A and Hemophilia B, total of 837 hemophilia cases were investigated.

Material and Methods: This research was cross-sectional and observational. Investigations were conducted on hemophiliac patients of various ages who had registered with the Hemophilia Patients Welfare Society in Rawalpindi. A thorough clinical and epidemiological history was gathered from the patient or the patient's accompanying parent or guardian using a pre-structured questionnaire. In accordance with test results and factor VIII and IX assay levels, patients were further divided into mild, moderate, and severe groups.

Results: One female patient of Hemophilia B is one of the important finding of this study. 0ut of total 837 patients investigated, 702 were of Hemophilia A and 135 were of Hemophilia B. 46.47% (389/837) belongs to the age group of >17 years. 476 (56.86%) were severe hemophiliacs, 269 (32.15%) were moderate and 92 (10.99%) were mild hemophilia patients. Knee was the most involved joint and it was observed in 331 (39.57%) patients.

Conclusion: The most common type of hemophilia is the severe form of hemophilia a (classic hemophilia). This study also found a female hemophilia B case which is the novel identification of this study. It is concluded that there is a need to start a national level diagnosis, prevention and care program for hemophilia patients.

Keywords: Hemophilia, bleeding, congenital, x-chromosome, knee joint.