Haemophilia

• hemophilia A is absent factor 8
• hemophilia B is absent factor 9

Hemarthrosis

• 2-3 bleeds in a short time may cause arthropathy
• iron absorbed by the synovium & causes damage by synovial cell release of enzymes against cartilage
• blood breakdown products also  destroy chondrocytes
• joint changes are similar to RA
• knee, ankle & elbow commonly affected

Treatment

• transfuse to get factor an ↑ in factor VIII
• ice & analgesia
• NWB until knee flexion returns to greater than 90°
• May aspirate major  hematoma
• May consider longterm prophylaxis
• Synovectomy – open versus scope

Muscle Bleeds

• Forearm, iliopsoas, quads & leg
• Rule out compartment syndrome
• Iliacus with femoral nerve palsy – get factor VIII up to 50% for 2 weeks
• In order to do a procedure like a fasciotomy need factor VIII to be 100%

Hemophilia Pseudotumor

• Bone or extraperiosteal blood
• Muscle bleed with fibrous capsule
• May expand with subsequent bleeds
• Commonly in hands & feet
• Excision is treatment

Fractures

• Non operative treatment –  should have VIII at 50% for 2-7 days
• Operative – need 80-100% preop & 30-50% for 1-2 weeks post-op