Publication:

Clinico-epidemiological profile of hemophilia with special reference to development of inhibitors in Indian scenario: a crosses sectional study at tertiary care centre in north India

Background Development of inhibitors is becoming a great challenge to clinicians in treating patients of Hemophilia. It is one of the most important complication affecting quality of life of patients and cost of treatment. The objective of the study was to study the prevalence of inhibitors in Hemophilia, its clinical presentation and factors (no. of exposure days, age of 1st presentation) affecting the development of inhibitors in Indian context.

Methods We conducted a cross-sectional study on 54 patients of severe Hemophilia admitted in our hospital. We assessed their treatment data and screened their samples for inhibitors by mixing study and confirmation done by Bethesda assay.

Results Out of 54 patients of severe Hemophilia 49 patients was of Hemophilia A and 5 of Hemophilia B. Out of these 49 patients 5 patients (10.2%) was positive for inhibitors. None of the HB is positive. Out of these 4 patients (80%) presented in their infancy. All patients had 20-50 exposure days. 24% patients had >50 exposure days, none of these develop inhibitor. Out of these 5 positive patients 4(80%) were low responders and 1 patient (20%) was high responder.

Conclusions In our study prevalence of inhibitor development is less compared to other western studies. This finding might be due to delayed initiation of factor concentrate treatment. In our study due cost of treatment bleeding prophylaxis is not given; only palliative treatment is available in our hospital. But considering small sample size and absence of genetic factors study it needs further study before reaching a final conclusion.