Friday, June 2, 2023
This activity is intended for hematologists, radiologists, pediatricians, and other clinicians caring for patients with joint bleeding due to hemophilia.
The goal of this activity is to review the clinical presentation and management of hemarthrosis in patients with hemophilia.
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An acute hemarthrosis is characterized by rapid joint swelling that may be preceded by a prodrome of tingling, reduced range of motion and pain.[50] The joint is often held in a flexed position, and the overlying tissues may be warm to palpation and extremely tender when touched or moved. Pain from bleeding into the ankles, knees or hips may make weight bearing impossible. In the absence of trauma, the presentation of acute joint bleeding is generally straightforward and does not require radiographic imaging.
On-demand therapy with a plasma-derived or recombinant FVIII or FIX concentrate is first-line treatment for acute bleeding events in patients with hemophilia. Dosing is largely based on uncontrolled, observational studies[51] and ranges from approximately 5[52] to 30 IU/kg[53] administered until bleeding stops. In the US Joint Outcome Study, acute hemarthrosis in the on-demand cohort was treated at a substantially higher dose and for an extended period of time: FVIII 40 IU/kg at the time of joint hemorrhage and 20 IU/kg at 24 and 72 h after the first dose. Parents were then encouraged to continue infusions of 20 IU/kg every other day, until joint pain and impairment of mobility had completely resolved. The benefits of this more aggressive therapy have yet to be determined in a rigorous way. An outstanding issue facing patients and clinicians alike is the objective determination of the cessation of joint bleeding. At this time, the patient’s or caregiver’s judgment must be relied on, as no objective test (i.e., ultrasonography or biomarker) has been demonstrated to be more informative.
Pain Control. Pain is a universal symptom of acute joint bleeding and chronic arthropathy, yet there is little evidence to guide therapy.[54] NSAID, such as ibuprofen, are effective analgesics,[55] but the potential for gastric bleeding is problematic.[56] Opioids, frequently used to relieve pain associated with chronic, noncancerous conditions,[57] have not been systematically evaluated for hemarthrosis, and concerns about abuse and addiction likely result in underuse of these drugs in the hemophilia population.[58] Little data are available regarding the efficacy of intra-articular corticosteroid injections to control hemophilic pain[59–61]
Rest, Ice, Compression & Elevation. Rest, ice, compression and elevation are generally recommended as an adjunct to factor replacement therapy during acute hemarthrosis,[62,63] but here again, the benefits of these conservative measures are not well established. Rest, with or without splinting, limits further injury and reduces pain, but prolonged rest poses a risk for muscle atrophy and contracture formation. While ice decreases pain, inflammation and tissue damage, it also slows blood flow, platelet function and enzymatic reactions, including conversion of prothrombin to thrombin.[64–66] Consequently, ice should be applied only after adequate factor replacement is administered (if factor concentrate is not readily available, ice can be used immediately). Swelling and edema are indicative of blood filling the joint space and inflaming the surrounding tissues. A compression bandage may help reduce soft tissue swelling, but it has little impact on bleeding. Finally, elevation of the affected limb to limit swelling is a good idea in theory but is not practical for young, active children.
Physiotherapy. Physiotherapy is an essential adjunct to factor replacement therapy in hemophilia patients with acute and chronic joint bleeding.[201] Hydrotherapy, for example, not only reduces pain but also decreases bleeding frequency and instability in target joints and improves range of motion and muscle girth.[67] Isometric and isotonic exercise, ultrasound, pulsed short-wave diathermy and transcutaneous electrical nerve stimulations are other modalities of physiotherapy used both acutely and chronically in the rehabilitation of hemophilia patients following hemarthrosis.[68]
Joint Aspiration. Arthrocentesis of intra-articular blood may be indicated in acute and profuse hemarthrosis to prevent long-term joint damage.[68] To be effective, joint aspiration should be performed within 2 days of bleeding onset, and before the procedure, factor concentrate must be administered to achieve 100% correction.[68] In general, however, the application of this procedure should be limited to episodes of extreme bleeding except for when the hip is involved in a young child where there is a risk of avascular necrosis.
Synovectomy. For individuals with pre-existing joint disease, prophylaxis may not sufficiently reduce the frequency of hemarthrosis, and, as mentioned, it cannot reverse progressive joint damage. When joint bleeding and synovitis are poorly controlled, synovectomy is effective in removing inflamed and hypertrophic synovium,[69] thus decreasing the propensity for repeated joint hemorrhage.[70,71] Surgical synovectomy, either open or arthroscopic, and radionuclide synovectomy, which involves the intra-articular injection of a radionuclide to induce fibrosis,[72] are all options for synovectomy. Success rates are similar for all three procedures, with synovectomy decreasing the frequency of joint bleeding by a reported 70–100%.[70,71]
