Hemophilia

Earliest feature- bluish subcutaneous bleeds Prolong bleed- cuts, dental extraction, surgery APTT may be normal in mild cases. Factor assay is diagnostic

Hemarthrosis
Mod-severe hemophiliacs develop 1st bleed as young as 2-5 years old Followed by hem-arthrosis-synovitis hemarthrosis cycle
Fe deposition -synovitis- hypertrophy Rich vascularization rebleed --- Target joint

Target joint
Reduced range of motion Muscle atrophy Cumulative joint deformity

Radionuclide Synovectomy

Advantage of Radionuclide Synovectomy

Advantages of radionuclide synovectomy vs surgery
Minimal factor requirement Several joints at once

Factor replacement concurrent with or prior to procedure to 50% N level Transfuse 8-12hrs later to reduce chance of bleed Admit/immobilize LE injections, all inhibitor pts 80% success rate