🩸 TOACS FCPS Station · Hemarthrosis in Hemophilia
📷 Clinical Photograph – Hemarthrosis (Knee)
🔍 Key clinical features:
- ✓ Swollen, warm, tender joint – most commonly knee, ankle, elbow
- ✓ Pain, limited range of motion – child refuses to bear weight
- ✓ Spontaneous or after minor trauma – hallmark of severe hemophilia (factor level <1%)
- ✓ Repeated episodes → chronic synovitis, target joint, hemophilic arthropathy
- ✓ Bleeding into joints – the most common site of bleeding in severe hemophilia
📋 Clinical scenario (examiner prompt)
A 6‑year‑old boy with a known history of severe hemophilia A (factor VIII <1%) presents with a swollen, painful, warm right knee that started this morning without any trauma. He is unable to bear weight on the leg and keeps it in slight flexion. He has a history of multiple joint bleeds, with the right knee being a target joint. He is on prophylaxis with factor VIII 30 IU/kg 3 times a week. His last dose was yesterday.
🧑⚕️ Examiner tasks · TOACS
1. Identify the diagnosis from the clinical image and context.
2. Describe the clinical features (hemarthrosis – acute joint swelling, pain, warmth, limited range of motion).
3. Explain the pathophysiology (factor VIII/IX deficiency → inadequate thrombin generation → bleeding into joints).
4. Discuss management (factor replacement, ICE, rest, and prevention of arthropathy).
🎯 Expected answers (for examiners)
- • Diagnosis: Acute hemarthrosis (joint bleed) in severe hemophilia A
- • Clinical features: Swollen, warm, tender joint; pain; limited range of motion; child refuses to bear weight; knee held in flexion
- • Pathophysiology: Factor VIII or IX deficiency → impaired thrombin generation → bleeding into synovial joints (most common site)
- • Treatment: Factor replacement (FVIII: 25-40 IU/kg, target 50-80%; FIX: 40-60 IU/kg), rest, ice, elevation, analgesia (avoid NSAIDs). If inhibitor present, use bypassing agents (rFVIIa or aPCC).
- • Prevention: Prophylaxis (FVIII 25-40 IU/kg 3x/week, FIX 40-60 IU/kg 2x/week) or emicizumab for hemophilia A
- • Complications: Recurrent bleeds → synovitis, target joint, hemophilic arthropathy (joint destruction)
• Most common bleeding site in severe hemophilia
• Common joints: Knee, ankle, elbow (in order of frequency)
• Target joint: ≥3 spontaneous bleeds in a joint within 6 months
• Treatment: Factor replacement to 50-80%, rest, ice, compression, elevation (RICE)
• Avoid NSAIDs – use acetaminophen or opioids for pain
• Prophylaxis prevents hemarthrosis and arthropathy
⚡ Quick FCPS‑style MCQ
A 6-year-old with severe hemophilia A presents with a swollen, painful, warm knee that started spontaneously. He is unable to bear weight. The most appropriate initial management is:
A. Factor VIII replacement (target 50-80%), rest, ice B. Ibuprofen for pain and inflammation C. Aspiration of the joint D. Immobilization only📌 Topic summary · Hemarthrosis in Hemophilia
Bleeding into a joint
Knee, ankle, elbow
Factor VIII/IX deficiency → impaired thrombin generation
Factor replacement (target 50-80%) + RICE
Prevents hemarthrosis and arthropathy
Hemophilic arthropathy
| Feature | Hemarthrosis |
|---|---|
| Definition | Acute bleeding into a joint (synovial joint) |
| Pathophysiology | Factor VIII or IX deficiency → inadequate thrombin generation → bleeding |
| Clinical presentation | Swollen, warm, tender joint; pain; limited range of motion; refusal to bear weight |
| Common joints | Knee (most common), ankle, elbow |
| Treatment (acute) | Factor replacement (FVIII 25-40 IU/kg, target 50-80%; FIX 40-60 IU/kg), RICE (rest, ice, compression, elevation) |
| Pain management | Acetaminophen, opioids; avoid NSAIDs (ibuprofen, naproxen) |
| Prophylaxis | FVIII 25-40 IU/kg 3x/week; FIX 40-60 IU/kg 2x/week; emicizumab for hemophilia A |
| Target joint | ≥3 spontaneous bleeds in a joint within 6 months |
| Complications | Chronic synovitis, hemophilic arthropathy, joint destruction |