Study finds muscle loss common in hemophilia A with joint bleeds

Ultrasound better for diagnosing sarcopenia than testing body composition

Margarida Maia, PhD avatar

by Margarida Maia, PhD |

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Illustration of the inside of a muscle.

Sarcopenia, or loss of muscle mass and strength, may occur in as many as half of adults with severe hemophilia A who experience recurrent joint bleeds, according to a small study in Turkey.

In addition, measuring muscle mass in the thighs with ultrasound, which is a noninvasive imaging test that uses sound waves, is better for diagnosing sarcopenia than measuring whole body composition, and offers a more accessible way for timely interventions, researchers note.

The study, “The frequency of sarcopenia in haemophilia patients: Effects on musculoskeletal health and functional performance,” was published in the journal Haemophilia.

Hemophilia A occurs when factor VIII, a protein that is needed for the blood to clot properly, is faulty or missing. As a result, people with hemophilia A may experience heavy, prolonged bleeds occurring either spontaneously or following an injury or surgery.

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Sarcopenia can develop in later stages of joint disease

Bleeding into the joints can cause them to swell or become damaged and limit their range of motion. Sarcopenia can also develop in the later stages of joint disease and further worsen these problems, as weakened muscles cannot support the joints as well, making movement harder.

In the study, researchers looked at how common sarcopenia was in 30 men diagnosed with severe hemophilia A who had recurrent joint bleeds as compared with 26 healthy men, and how sarcopenia affected joint health. The mean age was similar between the two groups (35.4 vs. 32.8 years).

On average, patients experienced 25 joint bleeds a year. The joints most affected by recurrent bleeds were those in the knees, followed by the elbows and ankles. Four patients (13.3%) had undergone orthopedic intervention. Eleven (36.7%) had a history of synovectomy, a procedure used to treat joint inflammation.

A modified Sonographic Thigh Adjustment Ratio (STAR) was used as a measure of muscle mass. It was calculated by finding the average thickness of the rectus femoris and vastus intermedius (two muscles in the front of the thighs) between the left and right sides, obtained by ultrasound, and dividing it by the body mass index.

The mean body mass index, a measure of body fat based on weight and height, was similar between the two groups (27.3 vs. 26), but mean STAR scores were significantly lower in patients than in healthy men (1.42 vs. 1.94). Based on STAR scores, 15 patients (50%) had sarcopenia.

The researchers also used bioelectrical impedance, which measures body composition based on how fast an electrical current travels through the body, as a way to diagnose sarcopenia. However, when using this method, two patients (6.6%) met the criteria for sarcopenia.

In all patients with sarcopenia, regardless of how it was diagnosed, muscle strength and functional performance were below normal. Functional performance was assessed by how far and fast patients could walk, or how fast they could stand up from a chair.

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‘We believe that ultrasound is an excellent diagnostic tool for sarcopenia’

“For the diagnosis of sarcopenia, decreased total and/or regional muscle mass together with decreased muscle strength and/or low physical performance are required,” the researchers wrote. “We believe that ultrasound is an excellent diagnostic tool for sarcopenia.”

The modified STAR was strongly linked to the Hemophilia Early Arthropathy Detection-Ultrasonography score, a measure of joint disease, as well as to the Hemophilia Joint Health Score. For bioelectrical impedance, the link was weak.

Similar findings were obtained when looking at the link between the modified STAR and hand or knee muscle strength; measures of functional performance like walking speed; or the time taken to complete five repetitions of sitting and standing up.

“The routine use of STAR scoring … could lead to earlier diagnosis and more effective interventions against sarcopenia, such as exercise, protein, calcium, vitamin D, creatine [a source of energy in muscles] supplements, and lifestyle modifications,” the researchers wrote.