FREQUENCY OF INHERITED PLATELE FUNCTION DISORDERS–ARMED FORCES INSTITUTE OF PATHOLOGY EXPERIENCE

Abstract

Objective: To determine the frequency and clinical features of inherited platelet function disorders diagnosed at Armed Forces Institute of Pathology.

Study Design: Cross sectional study.

Place and Duration of Study: Department of Haematology, Armed Forces Institute of Pathology, Rawalpindi, from Feb 2017 to Aug 2017.

Methodology: All patients with history of bleeding from multiple sites or requiring transfusion were selected. Initial tests performed included complete blood counts, peripheral film and bleeding time. When bleeding time was prolonged, prothrombin time and activated partial thromboplastin time were done to rule out Von Willebrand disease and Fibrinogen deficiency.

Results: A total of 172 patients were analyzed during study period.Inherited platelet function disorders were diagnosed in 24 (13.9%) patients, 111 (64.5%) patients had inherited coagulopathies. 3 (1.8%) patients showed inconclusive results while no inherited bleeding disorder was detected in 34 (19.8%) patients. Among platelet function disorders, Glanzmann thrombasthenia was diagnosed in 12 (50%) patients, Bernard soulier syndrome (BSS) in 11 (45.8%) patients and Storage pool disorder in 1 (4.1%) patient.

Conclusion: Glanzmann thrombasthenia was the most commonest inherited platelet function disorders. Diagnosis requires platelet aggregation studies.