FREQUENCY OF HEMOPHILIA A AND SEVERITY OF THE DISEASE AT PRESENTATION
Abstract
INTRODUCTION: Hemophilia A or Classic Hemophilia is a rare X-linked recessive bleeding disorder due to deficiency of factor VIII. The existence of such bleeding disorder is known for centuries.
OBJECTIVE: To determine the frequency of hemophilia A and severity of disease at presentation among patients presenting with highly suspected clinical features.
METHODOLOGY: This was a descriptive cross-sectional study carried out in the Department of Hematology, Post Graduate Medical Institute, Hayatabad Medical Complex, Peshawar from April 15th 2012 to March 30th 2013. 245 patients with highly suspected clinical features like joint bleeding, epistaxis, hematuria, unusual post trauma bleed, history of spontaneous bleeding and having prolongation of APTT were evaluated. All age group and both genders were included in the study. Patients with known coagulation factor deficiencies and those on anticoagulant therapy were excluded from the study. Factor VIII assay was carried out on Stago STA Compact Haemostasis Analyser. Results were interpreted in percentage (%) activity.
RESULTS: Out of these 245 patients 221 (86.1%) were male while 34 (13.9%) were female. The overall male to female ratio was 6.5: 1. Majority of patients 167(68.1%) age was ranged from 0-14 years, while 78(31.9%) were in the age range of above 14 years. Out of these 245 patients, 79(32.2%) were found to be Hemophilia A (factor VIII deficient). Among these 79 Hemophilia A patients 50(63.3%) were having severe Hemophilia A, 21(26.6%) moderate Hemophilia A and 8(10.1%) were mild Hemophilia A. Mean age for Hemophilia A in years was 10.41 with standard deviation of 8.16.
CONCLUSION: Frequency of Hemophilia A in was 32.2% in highly suspected patients. Most of the patients (63.3%) among these were having severe form of the disease.
KEY WORDS: Hemophilia A, Factor VIII Deficiency, Mild, Moderate and Severe Forms.
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