Joint status of patients with nonsevere hemophilia A

Abstract

Background Joint bleeding in hemophilia may eventually lead to joint damage. In nonsevere hemophilia, joint bleeds occur infrequently. Currently, knowledge on the joint status of patients with nonsevere hemophilia using objective imaging is limited. Objective To investigate the joint status in patients with nonsevere hemophilia A. Methods This cross‐sectional study included patients with nonsevere hemophilia A aged 24–55 years. Joint status was assessed by magnetic resonance imaging (MRI) of the elbows, knees, and ankles and International Prophylaxis Study Group (IPSG) scores were calculated. Lifetime joint bleeding history was collected from medical files. The contribution of factors to joint outcome was explored using multivariable linear regression analysis. Results In total, 51 patients were included, of whom 19 (37%) had moderate and 32 (63%) had mild hemophilia. Patients had a median age of 43 years (interquartile range [IQR] 32–50), a median factor VIII activity of 10 IU/dl (IQR 4–16) and a median annual joint bleeding rate (AJBR) of 0.0 (IQR 0.0–0.2). Soft‐tissue changes (IPSG subscore > 0) in the elbows, knees, and ankles were present in 19%, 71%, and 71% of patients, respectively. Osteochondral changes (IPSG subscore > 0) in the elbows, knees, and ankles were present in 0%, 20%, and 35% of patients, respectively. In 14% of bleed‐free joints, hemosiderin depositions were observed. Age and AJBRs were most strongly associated with the IPSG score. Conclusion This study demonstrates that a substantial proportion of adults with nonsevere hemophilia has joint changes on MRI despite low joint bleeding rates.

Prophylaxis use of clotting factor replacement products in people with non‐severe haemophilia: A review of the literature

“…A more recent publication described the first magnetic resonance imaging (MRI) study of joints in 51 people with non‐severe HA, of whom only 2 out of 19 people with moderate HA received prophylaxis. 50 The median total HJHS score was 3 (IQR 2–7) for the total cohort and 7 (IQR 4–11) for those with moderate HA. Ankle joints were the most frequently and structurally affected with haemosiderin deposits seen in 34% of ankles, 3% of knees and 8% of elbows, and 14% of bleed‐free joints were observed to have haemosiderin deposits.…”

“… 50 In comparison, people with moderate HA from the MoHem study had a lower median total HJHS score of 2 (IQR 0–10), 9 although the MoHem cohort was younger (median age 28 and 43 years, respectively). 9 , 50 …”

“…Among eligible studies, treatment regimens were reported for 631/2769 (22.8%) and 676/2291 (29.5%) with mild or moderate HA, respectively. 9 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 50 A total of 550 of people with moderate HA were treated with factor replacement prophylaxis, although the dose, frequency and duration of the prophylaxis regimen was not always mentioned; the vast majority came from western Europe 3 , 9 , 13 , 16 , 17 , 18 , 24 , 50 and Australia. 28 One study reported short‐term (6–12 weeks) low‐dose prophylaxis in 66 children with HA and arthropathy, of whom 32 children had moderate disease severity.…”

Validation of the patients‐specific functional scale in patients with haemophilia

“…This bleeding disorder causes recurrent bleeding into joints and muscles, resulting in increased stress on the musculoskeletal system 2 . This is the leading cause of morbidity in patients with severe haemophilia 3 .…”

“…1 This bleeding disorder causes recurrent bleeding into joints and muscles, resulting in increased stress on the musculoskeletal system. 2 This is the leading cause of morbidity in patients with severe haemophilia. 3 Synovial hypertrophy, cartilage degeneration, structural alteration, joint dysfunction and pain are clinical manifestations caused by recurrent bleeding, which result in haemophilic arthropathy (HA).…”