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
DOI:
https://doi.org/10.18203/2349-3291.ijcp20162366Keywords:
Hemophilia, India, InhibitorsAbstract
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.
References
Kliegman RM, Stanton BF, GemeIII JWS, Schor NF, Behrman. Nelson textbook of pediatrics 19th edition. 2011. p. 1699-1702.
Witmer C and Young G. Factor VIII inhibitors in Hemophilia A: rationale and latest evidence.Ther Adv Hematol. 2013;4(1):59-72.
Jarres. Current Controversies in the Formation and Treatment of Alloantibodies to Factor VIII in Congenital Hemophilia A. ASH Education Book. December 10, 2011. Pp. 407-412.
Mathews V, Nair SC, David S. Semin Thromb Hemost. 2009;35(8):820-6.
Franchini M, Mannucci PM. Past, present and future of Hemophilia: a narrative review. Orphanet J Rare Dis. 2012;7:24.
Ghosh K, Shetty S, Kulkarni B. Development of inhibitors in patients with Hemophilia from India. Hemophilia. 2001;7(3):273-8.
Kreuz W, Ettingshausen CE, Auerswald G. GTH PUP Study Group. Epidemiology of inhibitors and current treatment strategies. Haematologica. 2003;88(6):EREP04.
Phadke S. Hemophilia Care in India: A Review and Experience from a Tertiary Care Centre in Uttar Pradesh. Indian J Hematol Blood Transfus. 2011;27(3):121-6.
Gouw SC, Bom JG, Berg HM. Treatment-related risk factors of inhibitor development in previously untreated patients with Hemophilia A: the CANAL cohort study. Blood. 2007;109(11):4648-54.
Dubey A, Verma A, Elhence P. Evaluation of transfusion-related complications along with estimation of inhibitors in patients with Hemophilia. A pilot study from a single center. 2013;7(1):8-10.
Astermark J. Immune tolerance induction in patients with Hemophilia A. Thromb Res. 2011;127(Suppl 1):S6-9.
Eshghi P, Mahdavi-Mazdeh M, Karimi M. Hemophilia in the developing count. ries: the Iranian experience. Arch Med Sci. 2010;6(1):83-9.