Orthopedics - Oleksa A.P. 2006

Joint diseases
Hemophilic arthritis

Hemophilic Arthritis occurs in male hemophilia patients who suffer from severely impaired Blood clotting. The underlying cause of the arthritis is frequent hemorrhaging into the joints that are least protected from minor trauma (such as the knees, elbows, and ankles).

According to S. A. Reinberg (1964), hemarthrosis is the most characteristic feature of hemophilia, occurring in 40–90% of patients.

Hemophilia and frequent joint hemorrhages are caused by a deficiency or absence in Blood Plasma of antihemophilic globulin (hemophilia A), plasma thromboplastin (hemophilia B), or its precursors (hemophilia C), as well as other less thoroughly studied blood components (Hageman factor, factor "X", and others).

Each episode of hemarthrosis is accompanied by pain due to joint distension with blood and manifests as significant joint Swelling (Fig. 314), local hyperthermia, and a restricted range of motion resulting from an antalgic protective contracture. Minor hemorrhages may resolve completely without a trace, but more frequently they lead to reactive synovitis. Overstretching of the Joint Capsule caused by recurrent hemorrhages and synovitis leads to the deposition of coarse-dispersed protein fractions on all joint structures, which then organize, scar, and even ossify, ultimately causing The Development of hemophilic deforming arthrosis (Fig. 315).

The joint becomes permanently deformed, with a thickened capsule and a limited range of motion. Typically, flexion arthrogenic contractures develop, which impair normal limb function.

During X-ray Examination of the joints, the severity of observed changes depends on the frequency of recurrent hemarthrosis and synovitis.

Initially, radiographic examination reveals no pathological Changes in the joint; however, as hemorrhages recur, oval epiphyseal defects characteristic of hemophilia appear against a Background of Osteoporosis, along with joint space narrowing and marginal osteophytes that flatten the articular surfaces. Joint ankylosis is extremely rare. Subperiosteal hemorrhages following minor impacts may occur, accompanied by their ossification (Fig. 316).

Diagnosing hemophilic joint involvement is straightforward, as it is based on the patient's history (increased bleeding in boys from early childhood even with minimal Skin injuries, recurrent hemarthrosis, hereditary hemophilia, laboratory blood tests, etc.) combined with clinical and radiological findings.

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Fig. 314. Right elbow joint in a patient with hemophilia A.

Fig. 315. Radiograph of hemophilic arthrosis of the knee joints.

Fig. 316. Ossified subperiosteal hematoma of the Femur in hemophilia A.

Treatment. In acute cases of hemophilic hemarthrosis, the limb should be immobilized to provide rest for the affected joint, and a pressure bandage, ice pack, or cold compress should be applied.

There is no need to perform joint aspirations, as the blood may reaccumulate after drainage, and bleeding from the puncture site is also a risk.

It is advisable to transfuse fresh donor blood of the same blood group, or native or fresh frozen plasma, which contain all antihemophilic components.

In cases of hemophilia, antihemophilic globulin, factor VIII and IX concentrates, and Fibrinolysis inhibitors are transfused.

For joint deformities, discordant contractures, pseudotumors resulting from subperiosteal or soft tissue hemorrhages, as well as surgical conditions, surgical interventions are required. In Ukraine, these are performed exclusively in specialized surgical antihemophilic departments of the Kharkiv and Lviv Research Institutes of Hematology and Blood Transfusion, where patients should be referred urgently via air medical services.