Haemophilic Arthropathy
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Direct answer
Haemophilic arthropathy is a progressive joint disease caused by recurrent bleeding into joints in people with haemophilia A or B. Repeated haemarthrosis produces synovitis, cartilage loss and subchondral cysts, most often at the knee, ankle and elbow. Early factor replacement and prophylaxis prevent the damage, while synovectomy and arthroplasty address established disease.
What you must remember
- Cause: deficiency of factor VIII in haemophilia A, the commoner type, or factor IX in haemophilia B, both X-linked recessive and so affecting males.
- Target joints: the knee is most often involved, followed by the ankle and elbow, because they bear load and bleed repeatedly.
- Mechanism: blood breakdown products such as haemosiderin deposit in the synovium, driving chronic synovitis that erodes cartilage.
- Radiology: early periarticular osteopenia and soft-tissue swelling progress to subchondral cysts, joint-space loss and deformity, graded by the Arnold-Hilgartner system.
- Early treatment: prompt factor replacement after a bleed, with rest and elevation, while NSAIDs and aspirin are avoided because they impair platelet function.
- Surgical options: radionuclide synovectomy for recurrent synovitis, open or arthroscopic synovectomy when that fails, and total joint replacement for end-stage disease.
- Complication: inhibitors to replacement factor make bleeds harder to control and mean surgery needs a bypassing agent.
From recurrent bleeds to a planned operation
A 16-year-old with severe haemophilia A has had seven knee bleeds this year, and the knee stays swollen and flexed despite factor infusions. Plain films show joint-space narrowing and cysts at the joint margins.
- Treat the acute bleed. Factor replacement is given at once with rest and elevation, and the joint is moved only after the bleed is controlled.
- Prevent recurrence. Regular prophylaxis with factor concentrate, aimed at keeping trough levels above a set threshold, reduces repeated joint bleeds and cartilage loss.
- Grade the joint. Clinical examination and radiographs graded by the Arnold-Hilgartner system, with MRI or ultrasound where needed, judge the extent of synovitis and cartilage damage.
- Attempt synovial control. Radionuclide synovectomy is considered for recurrent haemarthrosis with active synovitis, with surgical synovectomy held in reserve if it fails.
- Plan arthroplasty for end-stage joints. Deformed, painful joints are replaced by a combined orthopaedic and haematology team, since bleeding and wound complications are more frequent in this group.
- Cover surgery with factor. Factor levels are raised near 100 per cent before and early after major surgery and tapered over about two weeks, as per the haemophilia centre protocol, once inhibitor status is checked.
Where haemophilia traps the surgeon
Haemophilic joint questions turn on drug safety and perioperative planning.
- Trap: giving aspirin or an NSAID for the knee pain of a haemophiliac Correct: these impair platelet function and worsen bleeding, so analgesia uses paracetamol alongside factor cover.
- Trap: attributing recurrent spontaneous haemarthrosis in a young man to trauma alone Correct: repeated bleeding into the same joint indicates haemophilia and should trigger a factor level test.
- Trap: planning joint replacement without factor cover or inhibitor screening Correct: replacement in haemophilia needs perioperative factor and inhibitor assessment, otherwise wound bleeding and haematoma follow.
Frequently asked questions
Which joint is most commonly affected in haemophilic arthropathy?
The knee is the most commonly affected joint in haemophilic arthropathy, followed by the ankle and elbow. These joints bear load and bleed repeatedly, so they accumulate the most synovial and cartilage damage over time.
Why are NSAIDs avoided in haemophilia?
NSAIDs inhibit platelet cyclo-oxygenase and prolong bleeding, so they are avoided in patients with haemophilia, particularly around a haemarthrosis. Paracetamol is preferred for analgesia, with factor replacement treating the bleed itself.
What perioperative factor cover is needed before arthroplasty in haemophilia?
Factor levels are raised close to normal immediately around surgery and then tapered over about two weeks, as set by the haemophilia centre protocol. Inhibitor status is checked beforehand, because inhibitors change the choice of replacement or bypassing agent.
What is radionuclide synovectomy used for?
Radionuclide synovectomy uses an intra-articular beta-emitting isotope to destroy recurrent synovitis in early haemophilic arthropathy. It reduces bleeding without open surgery, and it is generally tried before open synovectomy or joint replacement is considered.
What is the Arnold-Hilgartner staging used for?
The Arnold-Hilgartner staging is a radiological system that grades haemophilic arthropathy from early periarticular osteopenia and soft-tissue swelling to late cysts, joint-space loss and deformity. It tracks progression and helps guide the choice between prophylaxis, synovectomy and arthroplasty.