- 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;
