Quantification and Frequency of Inhibitors in Congenital Coagulation Disorder Patients; An Experience at Tertiary Care Hospital

Abstract

Objective: To quantify and calculate the frequency of inhibitors in patients with congenital coagulation disorders using the Bethesda assay.

Methodology: A cross-sectional study was conducted in the Department of Hematology at Children’s Hospital Lahore, Pakistan, from September 7, 2016, to March 7, 2017. 350 Pediatric patients of 1-15 yrs of age of both genders having congenital coagulation disorders were selected in the study. Patients having Hepatitis B, C, chronic liver disease evidence, HIV or malignancies were excluded from the study. The Chi-square test was applied, and a P-value of ?0.05 was considered statistically significant.

Results: A total of 350 children were enrolled in the study, comprising 277 (79.1%) males and 73 (20.9%) females. The age of the children ranged from 1 to 15 years. Among them, 127 (36.3%) were diagnosed with Hemophilia A, 73 (20.9%) with Hemophilia B, 38 (10.9%) with rare bleeding disorders, and 112 (32%) with Von Willebrand disease. Sixteen (5.77%) patients developed inhibitors after receiving treatment, all of whom were males aged 1 to 4 years. The quantification of inhibitors in these patients was performed using the Bethesda Assay.

Conclusion: Coagulation factor inhibitors may develop against any coagulation factor, with FVIII (Haemophilia-A) being the most common target. The evaluation of coagulation factor inhibitors involves the Bethesda assay to measure inhibitor titers, which aids in treatment decision-making.