FREQUENCY OF BLEEDING DISORDERS DIAGNOSED AT ARMED FORCES INSTITUTE OF PATHOLOGY RAWALPINDI

Abstract

Introduction: Disorders of hemostasis can be divided into hereditary and acquired and can further be compartmentalized into deficiency of the procoagulant proteins and defects of the platelets and vasculature. There is scanty data on the frequency of bleeding disorders in Pakistan.
Objective: To determine the frequency of bleeding disorders diagnosed at Armed Forces Institute of Pathology, Rawalpindi (AFIP Rwp).
Study design: Descriptive study.
Setting and duration: Department of Hematology, AFIP Rwp from January 2006 to June 2009.
Materials and Methods: A total of 1836 patients of bleeding diathesis were included in the study. Hess test was done to investigate the vascular defects. Bleeding Time (BT) was done to screen platelet function defects. The ‘clotting screen’ and mixing studies were done to detect coagulation protein defects. Clot solubility test was performed to screen factor XIII deficiency.
Results: Out of 1836 patients of bleeding diathesis 435 (23.7%) were diagnosed vas having haemostatic defects. Out of these 435 patients 273 (62.8%) had coagulation factor deficiency, 81 (18.6%) had platelet function defects and 81 (18.6%) had vWF deficiency. Among the 273 coagulation factor deficiency patients, factor VIII deficiency was 121 (44.3%), factor IX deficiency 32 (11.7%), factor V deficiency 18 (6.6%), factor XIII deficiency 15 (5.5%), factor VII deficiency 12 (4.4%), factor X deficiency 9 (3.3%), factor I deficiency 8 (2.9%) and factor II deficiency was 3 (1.1%). Multiple factor deficiency was 55 (20.1%). No defects of vasculature were identified.
Conclusion: Coagulation factor deficiencies with factor VIII deficiency being the commonest are the most frequent bleeding disorders. Platelet function defects and vWF deficiency also comprise significant proportion of the bleeding disorders.