Hemophilia & Hemophilic Arthritis Causes and treatment

 Hemophilia & Hemophilic Arthritis- Any of the 14 coagulation factors may be deficient in patients with hemophilia, but musculoskeletal problems are found primarily in patients with deficits of factor VIII or factor IX. The other inherited coagulation disorders are uncommon and, more important, are characterized by mucosal hemorrhage; they rarely demonstrate hemorrhage into a joint. 

Hemophilic arthropathy begins with a hemarthrosis, particularly when two or three bleeding episodes occur in a joint within a short period. 

As blood inside the joint is catabolized, breakdown products must be absorbed by the synovium. Iron is the most damaging element. Synovial cells can absorb only a limited amount of iron; when that quantity is exceeded, the cell disintegrates and releases lysozymes that not only destroy articular cartilage but also inflame the synovial tissue. 

The result is a hypertrophic and hypervascular synovium that, in a person with a clotting deficiency, is fragile and tends to bleed easily. Thus begins the vicious cycle of recurrent hemarthrosis followed by more synovitis and joint destruction. 

Chondrocytes take up iron as well, and with excessive amounts of iron in the joint, the chondrocytes also disintegrate and release  lysozymes that destroy the cartilage matrix. Furthermore, the factory (the chondrocyte) also is destroyed. This combination of synovitis and primary articular cartilage erosion explains the rapid and severe arthropathy seen in hemophilic patients who do not have access to adequate replacement therapy. 

➧Radiographic changes in the early stages of hemophilic arthropathy are similar to those observed in rheumatoid arthritis. 

➦Erosions at the joint margin, 

➦Subchondral cysts

➦Subchondral irregularity

➦Widening of the intercondylar notch of the femur

➦Enlargement of the radial head 

These changes as the arthropathy progresses. With end-stage arthropathy, narrowing of the articular cartilage is obvious, but the subchondral bone is more sclerotic in hemophiliacs than in patients with rheumatoid arthritis . 

Transfusion to replace the missing clotting factor is most critical in the management of any hemarthrosis. Major joint bleeding should be followed by a period of prophylactic transfusion therapy. Aspiration of the joint can remove the bulk of blood and may reduce the amount of iron that must be absorbed. If hypertrophic synovitis develops, the patient may be treated with radiation synovectomy, arthroscopic synovectomy, or open synovectomy. 

The enlarged and incongruent radial head is most often also excised at the time of an elbow synovectomy. Total joint arthroplasty can be helpful with endstage arthritis.