Spectrum of Bleeding Disorders In Pakistan: A Cross-Sectional Study
Author(s): Muhammad Sohaib Asghar, Ayesha Asghar, Fatima Asghar, Mohammed Akram
This is a cross-sectional study conducted to determine the frequency and spectrum of bleeding disorders in different regions of Pakistan and to improve facilities to cure them. Samples were collected from different regions of Pakistan. There bleeding presentation and history of the family were recorded. 435 patients were selected from the different regions of Pakistan; 318 male (73%) and 117 female (26%). Laboratory analysis included Prothrombin time, Activated partial thromboplastin time, Bleeding time, platelet counts and fibrinogen assay were performed. According to their results, vWF:Ag, vWF:RCo, inhibitor screening, Bethesda test, urea clot solubility test, and platelet aggregation test were performed to diagnosed bleeding disorders in patients. Out of 435 patients, 283 constitute CBD, 80 diagnosed with RBD, and 72 remain undiagnosed. Patients diagnosed with 52% hemophilia A, 1.4% with its inhibitor, 8.4% with hemophilia B, 0.35% with its inhibitor, 3.88% with VWD, 33.5%with its type III. A rare bleeding disorder is diagnosed in 80 subjects with 20% fibrinogen, 16.25% FXIII, 15% FVII, 11.25% FV, 10% vitamin k dependent factors, 8.25% GT, 5% FXII and epinephrine, 2.5% FII and FX and 1.25% FV + FVIII, BSS and FXI deficiencies. Clinical bleeding episodes varied in patients with respect to its deficiencies include gum bleeding, menorrhagia, hematoma, bruises, umbilical cord bleeding, hemorrhagia, and hemarthrosis. Study shows that hemophilia A is more frequent than hemophilia B. vWD type III has a higher prevalence. In RBD, fibrinogen deficiency is the most common, followed by FXIII, FVII, and FV respectively.