A significant number of adult patients with hemophilia have joint changes on magnetic resonance imaging (MRI) despite having low joint bleeding rates, according to the results of a new study published in the Journal of Thrombosis and Haemostasis.

Joint injury due to bleeding is common in patients with hemophilia. However, patients with nonsevere hemophilia seldomly suffer from bleeding episodes. Zwagemaker and colleagues aimed to clarify joint status among patients with nonsevere hemophilia A.

The authors conducted a cross-sectional study in patients with nonsevere hemophilia A aged 24 to 55 years. They assessed joint status using MRI, particularly of the elbows, knees, and ankles; then, they calculated the International Prophylaxis Study Group (IPSG) scores and collected lifetime joint bleeding histories from the medical records. Multivariable regression analysis assessed the factors contributing to the final joint outcomes.

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Out of 51 patients, 37% had moderate hemophilia and 63% had mild hemophilia. The patients had a median age of 43 years, median factor VIII level of 10 IU/dL, and median annual joint bleeding rate of 0.0.

Soft tissue changes, defined by an IPSG subscore >0, were noted in the elbows, knees, and ankles in 19%, 71%, and 71% of patients, respectively. Osteochondral changes, also defined by an IPSG subscore >0, were present in elbows, knees, and ankles in 0%, 20%, and 35% of patients, respectively. There were hemosiderin depositions in 14% of bleed-free joints. 

The authors noted that age and median annual joint bleeding rate was strongly associated with the IPSG score. “This study demonstrates that a substantial proportion of adults with non-severe hemophilia has joint changes on MRI despite low joint bleeding rates,” the authors concluded.


Zwagemaker AF, Kloosterman FR, Hemke R, et al. Joint status of patients with non‐severe hemophilia AJ Thromb Haemost. Published online February 16, 2022. doi:10.1111/jth.15676