Clinical Manifestations: Hemophilic Arthropathy



- See: Extra-articular Manifestations

- Discussion:
    - manifests itself in one of three typical forms:
         - acute hemarthrosis;
         - subacute hemarthropathy;
         - chronic hemarthropathy;
    - severity of hemophilia A depends upon level of factor VIII clotting activity;
         - severe deficiency:
                - factor VIII activity level of < 1%;
                - pt characteristically bleeds into joints or muscles spontaneously or w/ minimal trauma;
         - moderate deficiency:
                - involved patients have between 1-5% factor VIII activity;
                - spontaneous hemorrhage is infrequent;
                - joint or muscle bleeds may occur with minor trauma;
         - mid deficiency:
                - have more than 5% factor VIII levels;
                - pts usually bleed only with more severe trauma or during surgery;
- Sites of Involvement:
    - most commonly affected joints: knee > elbow, ankle, shoulder, hip and wrist.
- Acute Hemarthrosis:
    - pain is the early and predominant symptom;
    - swelling, tenderness, warmth, and impaired mobility are common;
    - treatment:
           - replaceme missing clotting factor to 30-50% of normal levels;
           - compressive dressings, immobilization by splints, and joint aspiration, will decrease pain and reduce the incidence of recurrent bleeds.
           - after 1 to 2 days, ROM & strengthening exercises can begin;
- Subacute Hemarthropathy:
    - pt has synovial hypertrophy and frequent bleeds in the affected joint;
    - destruction of joint, atrophy of the surrounding muscles, & joint contractures begin during this stage;
    - x-rays:
           - osteopenia of the epiphyses assoc w/ synovial hyperemia;
           - squaring of epiphyses;
           - widening of intercondylar femoral notch;
           - cystic changes in subchondral bone;
    - treatment:
           - treatment is directed at reducing frequency of hemarthrosis and to correct contractures and muscle atrophy;
                  - this will diminish synovial hypertrophy & halt joint destruction;
           - synovectomy may be helpful.
- Chronic Hemarthropathy:
    - progression of hemarthropathy leads to further degenerative changes;
    - as articular cartilage becomes fibrillated and eroded, patient develops more pain, restricted motion & deterioration of function;
    - posterior subluxation of the tibia is frequently associated w/ chronic hemarthropathy of the knee.
    - x-ray changes:
           - irregular articular surfaces;
           - narrowing of cartilage space, osteophytes, and subchondral cysts;
    - treatment:
         - treatment involves correction of deformity, maintenance of correction, improving motion, and strengthening of muscles



Original Text by Clifford R. Wheeless, III, MD.

Last updated by Data Trace Staff on Wednesday, August 22, 2012 12:36 pm