Original Articles
Assessment of prevalence of clotting factor inhibitors in patients with haemophilia | |
Dr. Priyanka Tiwari | |
Background:X-linked genetic changes in the coagulation factor production—which is crucial for preserving hemostasis—cause hemophilia, a bleeding disorder. The present study was conducted to assess prevalence of clotting factor inhibitors in patients with hemophilia. Materials &Methods:54 cases of hemophilia of both genderswas recruited. Based on the factor VIII bioassay and bleeding profile, phenotype analysis was carried out.In order to undertake mixing-based inhibitor screening, patient plasma and pooled normal plasma (PNP, which was obtained from 20 healthy donors) were mixed in a 1:1 ratio. Results:All 54 patients were males. Type of haemophilia was A in 47, B in 5 and Hemophillia+ Von Willebrand disease in 2 patients. Factor VIII activity was mild in 7, moderate in 21 and severe in 26 cases. The difference was significant (P< 0.05). Mixing based inhibitor screening was positive in total 15 patients. Bethesda assay confirmed 10 cases with presence of inhibitor out of 15 cases. Conclusion: Since follow-up investigations are more expensive and time-consuming than basic screening tests, mixing tests are a crucial first step in the evaluation of inhibitors in hemophilia cases. |
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