Inherited platelet function disorders versus other inherited bleeding disorders: an Indian overview

Abstract

Inherited deficiencies of plasma proteins involved in blood coagulation generally lead to lifelong bleeding disorders, whose severity is directly proportional to the degree of factor deficiency. Platelet and other coagulation factors play an important role in the haemostasis mechanism. We attempted to study the prevalence of inherited platelet function disorders (PFDs) and correlate with other coagulation factor disorders in the Indian population. Patients with PFDs and other coagulation factor disorders who presented at our hospital during the 5 year period (from January, 2001 to December, 2005) were the subjects of the study. A total of 1576 patients were diagnosed to have congenital bleeding disorders including PFDs, von Willebrand disease, haemophilia A and B and rare coagulation disorder cases. Haemophilia A (HA) was the most common and was seen in 52.31% of the patients followed by total PFDs seen in 27.77% of the patients. Based on severity of the disease, the results of PFDs were highly significant when compared to haemophilia and von Willebrand disease (VWD) (p=0.000). Severity was found higher in HA (77.8%) followed by HB (69.6%) and was found lower for PF3 availability defect (9.0%). It has been concluded that the prevalence of PFDs is relatively low as compared to coagulation factors related disorder and also it has been established that type-1 VWD is relatively less frequent in India as compared to the West.