Haematological- Haemophilia

Question 1

Def.

Answer 1

• deficiency of clotting factors
• sex linked (M>F)
• eventual destruction of underlying bone due to continual bleeding (c.f. mechanism with chronic inflammation ie. RA)

Question 2

Radiol Fx.

Answer 2

• Knee most common site ( joint space maintained until late stages.)
• Grossly enlarged femoral condyles (megacondyle)with osteoporotic medullary region
• irregular and flattened weight bearing surfaces.
• May see soft tissue swelling with haemarthrosis, overlying fascial planes.
• Osteoporosis and poorly defined articular cortex.
• May also see joint disorganisation with:
  • complete loss of joint space,
  • articular fragmentation,
  • sclerosis,
  • osteophytes
  • bony misalignment.

N.B. Soap Bubble appearance of bone possible

* DDx 
	* ABC / SBC
	* GCT
	* Osteoblastoma
	* Fibrous Dysplasia

• Ankylosis also possible as rare late-term complication