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HEMOPHILIA. John O’Sullivan 24 May 2001. Outline. Definition Epidemiology Diagnosis Factor Replacement Hemophilic Arthropathy Treatment Other Topics. Hemophilia. Disorder of hemostasis, a coagulopathy Hemophilia A - Factor VIII deficiency Hemophilia B – Factor IX deficiency

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hemophilia

HEMOPHILIA

John O’Sullivan

24 May 2001

outline
Outline
  • Definition
  • Epidemiology
  • Diagnosis
  • Factor Replacement
  • Hemophilic Arthropathy
  • Treatment
  • Other Topics
hemophilia3
Hemophilia
  • Disorder of hemostasis, a coagulopathy
  • Hemophilia A - Factor VIII deficiency
  • Hemophilia B – Factor IX deficiency
  • Prevalence: 13.4 cases per 100,000 males
  • Incidence: 1 in 5032 live male births
diagnosis
Diagnosis
  • Atypical bleeding at circumcision or bruising at neonatal vaccines
  • Toddlers with lip bleeding or unusual bruising when learning to walk
  • Hx of affected males on mother’s side
  • Elevated PTT
  • Factor assays
factor deficiencies
Factor Deficiencies
  • Severe: <1%
    • Hemarthrosis with minimal trauma or ADLs
  • Moderate: 1 to 5%
    • Intermediate symptoms with fewer hemarthroses
  • Mild: >5%
    • Joint bleeds rarely develop except with significant trauma
factor replacement
Factor Replacement
  • For routine muscle or joint bleed give 25 U/kg to maintain levels >1% for 48 hours
  • Preoperatively:
    • Screen for inhibitors
    • Elevate F VIII levels to >100% 1 hr preop
    • Maintain at 60%
    • If vigorous PT required, transfuse to 50% pretreatment
preoperative evaluation
Preoperative Evaluation
  • Accurate diagnosis
  • Accurate quantification
  • Adequate reserves of factor
  • Lab must be able to perform assays
  • HIV, Hepatitis status
hemophilic arthropathy
Hemophilic Arthropathy
  • As blood is catabolized, it is absorbed by synovium
  • Iron is toxic to cells – synovial cells disintegrate releasing lysosomes which destroy cartilage and inflame synovium
  • Hypertrophic, hypervascular synovium
  • Chondrocytes also affected
  • FIBROSIS
radiographic changes
Radiographic Changes
  • Like RA
    • Soft tissue swelling
    • Osteopenia
    • Overgrowth of epiphysis
    • Erosions
    • Cysts
treatment
Treatment
  • Prophylactic transfusions must be started at age 2 to 3
    • Need central access
    • Risk of bacteremia
    • Costly
  • Aspiration: lowers blood load
treatment16
Treatment
  • Arthroscopic synovectomy
  • Radiosynovectomy
    • 32P chromic phosphate
    • 75% good to excellent results
slide17
Knee
  • Arthroscopic synovectomy
  • Open debridement
  • TKA
  • Arthrodesis
slide18
ELBOW
    • Open synovectomy with excision of radial head
      • Contraindicated in children
  • ANKLE and SUBTALAR JOINT
    • Arthrodesis
slide19
Hip
  • Two modes of onset
    • Rapid progression due to increased intracapsular pressure
    • Chronic synovitis
  • THA
    • 33% aseptic failure rate
shoulder
Shoulder
  • Abandoned open synovectomy
  • Arthroscopy limited
  • Arthrodesis
  • Arthroplasty
other topics
Other Topics
  • Muscle bleeds
  • Pseudotumor
  • HIV