A Clinical and Therapeutic Appraisal in Hemophilia-A

Abstract

The hemorrhagic diseases present a complex problem in medicine, for they are often difficult to diagnose and troublesome to manage. Understanding of structure of various coagulation factors and its genetic control, biological and immunological properties has revolutionized the management of coagulation disorders. Hemophilia A occupies a unique position among the hemorrhagic diseases. The present study centers on the critical evaluation of Hemophilia A patients. Attempt was also made to follow the cases carefully in order to study the natural history of disorder and to assess the response on treatment. The major clinical presentation of our patients was due to bleeding into Joints. Knee joint was the most common site of involvement, a major source of distress, incapacitation, and was showing gross deformity in 25% of cases. Recurrent gum bleeding, was a universal complaint and responsible for a sizeable number of hospital visits by hemophiliacs, surpassed only by joint problems in total burden on hemophilic patients. All the hemophiliacs had prolonged KCCT. WBCT was normal in 35% of cases. Thromboplastin generation test confirmed the type of Hemophilia. Factor VIII is expansive and financial constrain was the main limiting factor in treatment of Hemophiliacs. Inj. Factor VIII was given for episodes of bleeding into joints, bleeding into muscle, traumatic bleeding and intracranial bleeding. Acute hemarthroses was treated with factor transfusion and immobilization. For chronic hemophilic arthropathy emphasis was focused on physiotherapy specially in form of active exercises. Difficulty was experienced in continuing the programme because of super imposed episodes of acute bleeding into joint.