The X-linked bleeding disorder caused by deficiency of coagulation factors VIII and IX. Presnt with hemarthrosis, hemartomas, and delayed post operative and post-traumatic bleeding. Mild cases need intervention only after trauma or surgery. More severe cases with 2-4 spontaneous bleeds every month need regular management

All bleeds need in patient therapy. Treatment should be started early. Symptoms often precede obvious bleeding. Therapy should never wait for elaborate diagnostic work up. A hematologist should supervise the management. Cryoprecipitate 4-8 units twice daily the first day followed by 24 units daily in 2 doses subsequently until bleeding stops. OR Factor VIII concentrate 15 units/kg body weight every 12 hours for 3-5 days for mild bleeding and 39 units/kg body weight every 12 hours for 8-10 days for severer bleeds. Home therapy with Factor replacement will allow out-patient therapy for minor bleeds. Preventive measures include good dental care to avoid dental extractions to extent possible, avoidance of trauma, avoidance of IM injections, and avoidance of aspirin and NSAIDS.