Abstract
Background: Deficiency of factor VIII (FVIII) (Hemophilia A) and factor IX (Hemophilia B) are the most frequent coagulation defects. The incidence of hemophilia A varies in different regions of India.

Aims and Objectives: The present study was done to classify hemophilia A patients by estimating FVIII levels in them and to describe the clinical profile of the patients attending a tertiary care hospital in southern part of India, Kerala.

Materials and Methods: A hospital-based cross-sectional study was done on patients with hemophilia A, attending Department of Medicine and Department of Pediatrics from March 2012 to September 2013. Demographic and clinical details were collected using per forma and quantitative assessment of FVIII levels were done using semi-automated blood coagulation analyzer.

Results: Out of 90 cases studied, majority of the patients had severe hemophilia (85.6%), followed by mild (10%) and moderate hemophilia (4.4%). Positive family history was seen in 67.8% patients. Median age of the patients at diagnosis was 1 years of age. Most common clinical presentation was found to be hemarthrosis (84.4%) followed by bleeding into muscle (56.7%). Knee joint (75.6%) was the predominantly affected joint. Bony ankylosis (30%), intracranial bleed (14.4%), and retroperitoneal bleed (24.4%) were the most common complications noted. Bleeding manifestations such as gum bleed (24.4%), epistaxis (12.2%), hematuria (37.8%), and ecchymosis (15.6%) were also reported among them.

Conclusion: Proportion of severe hemophilia among hemophilia A patients was found to be higher in number than in other parts of the country. Serious complications can result from bleeding into the joints, muscles, brain, or other internal organs. Therefore, promotion of low cost, regular availability of factor concentrates, prophylactic factor replacement, establishing comprehensive care center, and regular training of medical and paramedical staff will help in reducing the complications due to this disease.

Key words: Clinical Profile; Complications; Factor VIII; Hemophilia A; Severe Hemophilia