Preventive therapy may lead to less joint damage in hemophilia patients

Real-world study finds joint health is worse in hemophilia A vs. B

Margarida Maia, PhD avatar

by Margarida Maia, PhD |

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A person reclines on a couch with one leg elevated and a bag of ice on the knee.

A real-world study in Spain found that, among hemophilia patients, those with hemophilia A may have have worse joint health — and more joint damage, particularly in the ankles — than those with hemophilia B.

Importantly, though, such damage appeared to be less frequent for individuals on regular preventive treatment, regardless of the type of hemophilia the patient had.

“These data support haemophilia recommendations to provide patients with [prophylaxis, or preventive therapy] regiments over [on-demand treatments] to impede joint bleeding,” which can lead to damage, the researchers wrote, noting that preventive therapy “is the most successful strategy in preserving joint health independently of haemophilia type.”

The team also noted that regular check-ups using ultrasound could help doctors detect joint damage early and provide tailored treatment plans to people with severe hemophilia A or B to help improve how well their joints work.

The study, “A post hoc comparative real-world analysis of HEAD-US score for joint health assessment of patients with severe haemophilia A and B in Spain,” was published in the journal Haemophilia.

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Worse joint damage seen in ankles in hemophilia A patients

Hemophilia occurs when blood fails to clot properly due to the lack or malfunction of a clotting protein in the body. In hemophilia A, the faulty or missing clotting protein is factor VIII, whereas in hemophilia B, it is factor IX.

As a result of poor clotting, people with hemophilia experience heavy, prolonged bleeding episodes that can occur spontaneously or following surgery or an injury. Repeated bleeding into the joints can cause hemophilic arthropathy, or permanent and irreversible joint damage.

Ultrasound scans can be used to detect many of the characteristics of such permanent damage, including synovial hypertrophy — a thickening of the synovial membrane, a thin barrier that lines the inside of the joints — and erosion of the joint cartilage.

Now, researchers from institutions across Spain used a type of ultrasound called HEAD-US — fully known as Hemophilia Early Arthropathy Detection with Ultrasound — to compare joint damage in people with hemophilia A and hemophilia B. A higher HEAD-US score reflects worse joint health.

The team specifically focused on patients’ left and right elbows, knees, and ankles.

Their study included 95 individuals with severe hemophilia A and 41 with severe hemophilia B from 12 medical centers across Spain. The patients’ mean age at the time of the study was similar (35.2 vs. 32.7 years), as was their mean age at the time of diagnosis (1.4 vs. 1.6 years).

The proportion of people who reported a so-called target joint — that is, a joint in which there was repeated bleeding — was similar between those with hemophilia A and hemophilia B (66.3% vs. 65.9%). In hem A, the ankles were most frequently affected, whereas in hem B, it was the knees.

Patients with hemophilia A had more joint damage than did those with hemophilia B. On average, HEAD-US scores for the left and right elbows were about 1.8 points higher in hemophilia A patients; for the left and right ankles, the scores were about 2.3 points higher.

The proportion of people who scored zero in joint damage was lower for those with hemophilia A than for those with hemophilia B (6.3% vs. 22%).

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Mild to moderate or severe synovial hypertrophy was detected in up to 47.4% of the joints. In the left ankle, but not in any of the other joints, synovial hypertrophy was nearly twice as common in people with hemophilia A compared with hemophilia B (47.4% vs. 26.8%).

Destruction of the joint cartilage and complete bone damage also were detected in about four times as many people with hemophilia A as in those with hemophilia B, indicating “substantially increased joint damage,” the researchers wrote.

Osteochondral damage, which involves both the cartilage and the underlying bone, occurred more frequently in patients who received on-demand treatment or tertiary prophylaxis versus primary or secondary preventive care.

Primary prophylaxis refers to preventive treatment given before any joint damage has occurred, whereas secondary prophylaxis is given after patients experience repeated joint bleeding, but before the onset of joint disease. Tertiary prophylaxis refers to regular preventive treatment that is started following the onset of joint disease.

Continuing [ultrasound] monitoring for the early detection and management of [joint] abnormalities, as well as more efficiently tailored therapies should be warranted.

“Increased disease activity and articular damage were found in joints of patients with severe disease who were either treated on demand or tertiary prophylaxis, regardless of the type of haemophilia,” the researchers wrote.

Conversely, patients who received regular primary or secondary prophylaxis had less synovial hypertrophy and joint damage than those who were receiving on-demand treatment or tertiary prophylaxis.

“Continuing HEAD-US monitoring for the early detection and management of intra-articular [joint] abnormalities, as well as more efficiently tailored therapies should be warranted,” the researchers concluded.