Journal of Blood Disorders & Transfusion

Abstract

The Hemophilic Joints

E. Carlos Rodriguez-Merchan

Recurrent hemarthroses in childhhood before the initiation of primary prophylaxis will result in joint damage after a decade or later. The best way that we have today to protecting against hemophilic arthropathy is primary prophylaxis. The decision to institute early full prophylaxis by means of a port (central venous access device) has to be balanced against the child’s bleeding tendency, the family’s social situation and the experience of the specific hemophilia center. The reported complication rates for infection and thrombosis have varied considerably from center to center. Risk of infection can be reduced by repeated education of patients and staff, effective surveillance routines and limitations on the number of individuals allowed to use the device. In discussing options for early therapy, the risks and benefits should be thoroughly discussed with the parents. From a practical point of view, primary prophylaxis together with synovectomy (radioactive, chemical, arthroscopic, or open) to avoid joint bleeding, can help halt hemophilic synovitis. Radiosynovectomy is a relatively simple, virtually painless and inexpensive treatment for chronic hemophilic synovitis, even in patients with inhibitors and must be the best choice for patients with persistent synovitis (which must be confirmed by US and/or MRI). Arthroscopic joint debridement may be indicated in younger hemophiliacs when there is relative preservation of the joint architecture and congruity, after the failure of three radiosynovectomies with a 6-month interval, and when joint replacement is not considered to be a viable alternative. Total joint arthroplasty should be indicated in hemophiliacs with severe joint pain and disability, including those who are HIV-positive. Hemophilia is a risk factor for infection after total joint arthroplasty. An extended period (2-3 weeks) of adequate hemostatic function is necessary to achieve normal healing after orthopedic procedures in hemophilia.