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Published on: December 27, 2017
Inherited platelet function disorders versus other inherited bleeding disorders: an Indian overview
Firdos Ahmad1, Meganathan Kannan, Ravi Ranjan
1Department of Haematology, IRCH Building 1st floor, All India Institute of Medical Sciences, Ansari Nagar, New Delhi-110 029, India.
Thrombosis Research
|September 14, 2007
Summary
Inherited platelet function disorders (PFDs) are less common than coagulation factor disorders in India. This study found PFDs in 27.77% of congenital bleeding disorder patients, with varying severity.
Area of Science:
- Hematology
- Genetics
- Internal Medicine
Background:
- Inherited plasma protein deficiencies cause lifelong bleeding disorders.
- Platelet and coagulation factors are crucial for hemostasis.
- The prevalence and characteristics of inherited platelet function disorders (PFDs) in India require further investigation.
Purpose of the Study:
- To determine the prevalence of inherited platelet function disorders (PFDs) in the Indian population.
- To correlate PFDs with other coagulation factor disorders.
- To assess the severity of PFDs in comparison to other bleeding disorders.
Main Methods:
- Retrospective analysis of 1576 patients with congenital bleeding disorders from January 2001 to December 2005.
- Inclusion criteria: Patients diagnosed with PFDs, von Willebrand disease, hemophilia A or B, and rare coagulation disorders.
- Data collected on disorder type, prevalence, and disease severity.
Main Results:
- Hemophilia A (HA) was the most frequent (52.31%), followed by PFDs (27.77%).
- PFDs showed highly significant results regarding disease severity compared to hemophilia and von Willebrand disease (VWD) (p=0.000).
- Severity was highest in HA (77.8%) and Hemophilia B (HB) (69.6%), and lowest in PF3 availability defect (9.0%).
Conclusions:
- Inherited platelet function disorders (PFDs) are less prevalent than coagulation factor disorders in India.
- Type-1 von Willebrand disease (VWD) appears less frequent in India than in Western countries.
- Further research is needed to understand the full spectrum of inherited bleeding disorders in the Indian population.
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