TY - JOUR
T1 - Joint status of patients with nonsevere hemophilia A
AU - Zwagemaker, Anne-Fleur
AU - Kloosterman, Fabienne R.
AU - Hemke, Robert
AU - Gouw, Samantha C.
AU - Coppens, Michiel
AU - Romano, Lorenzo G. R.
AU - Kruip, Marieke J. H. A.
AU - Cnossen, Marjon H.
AU - Leebeek, Frank W. G.
AU - Hutten, Barbara A.
AU - Maas, Mario
AU - Fijnvandraat, Karin
N1 - Funding Information: S. C. G. has received an unrestricted research grant from Sobi. M. C. has received financial support for research from Bayer, CSL Behring, Daiichi Saknyo, Portola/Alexion, Roche, Sanquin Blood Supply, and UniQure and consultancy or lecturing fees from Bayer, CSL Behring, Medcon International, MEDtalks, Novo Nordisk, Pfizer, and Sobi. L.G. R. R. received the Sobi Young Investigators Award 2020 and a travel grant from Sobi (2019). M. J. H. A. K. has received unrestricted financial support for research from Bayer, Daiichi Sankyo, and Sobi and lecturing fees from Bayer en Roche, all payments to the institute. M. H. C. has received investigator‐initiated research and travel grants as well as speaker fees over the years from the Netherlands Organisation for Scientific Research (NWO), the Netherlands Organization for Health Research and Development (ZonMw), the Dutch “Innovatiefonds Zorgverzekeraars”, Baxter/Baxalta/Shire/ Takeda, Pfizer, Bayer Schering Pharma, CSL Behring, Sobi Biogen, Novo Nordisk, Novartis, and Nordic Pharma, and has served as a steering board member for Roche, Bayer, and Novartis. All grants, awards and fees go to the Erasmus MC. F. W. G. L. received unrestricted research grants from CSL Behring, Shire/Takeda, SOBI, and uniQure. He is a consultant for CSL Behring, Takeda, Biomarin, and uniQure, of which the fees go to the University. He received travel support from SOBI. He is DSMB member of a study sponsored by Roche. The institution of K. F. has received unrestricted research grants from Sobi, Pfizer, CSL Behring, and Novo Nordisk and her institution received consultancy fees from Grifols, Takeda, Novo Nordisk, and Roche. A. .Z, F. R. K., R. H., B. A. H., and M. M. have no potential conflicts of interest. Funding Information: This work was supported by grants from Novo Nordisk, CSL Behring (Prof. Heimburger Award 2016) and Bayer (Outcomes Research Award of the Bayer Hemophilia Awards Program 2015). The funding sponsors had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; and preparation, review, and approval of the manuscript. Funding Information: The authors thank Sandra van den Berg and Raschel van Luijk (Amsterdam UMC, Amsterdam, the Netherlands) for their technical support and help in conducting the MRI scans. In addition, the authors thank Cees Nooij and Wypke de Boer for their training on assessment of the Haemophilia Joint Health Score. This work was supported by grants from Novo Nordisk, CSL Behring (Prof. Heimburger Award 2016) and Bayer (Outcomes Research Award of the Bayer Hemophilia Awards Program 2015). The funding sponsors had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; and preparation, review, and approval of the manuscript. Publisher Copyright: © 2022 The Authors. Journal of Thrombosis and Haemostasis published by Wiley Periodicals LLC on behalf of International Society on Thrombosis and Haemostasis.
PY - 2022/5
Y1 - 2022/5
N2 - 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.
AB - 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.
KW - hemarthrosis
KW - hemophilia A
KW - joint diseases
KW - joints
KW - magnetic resonance imaging
UR - http://www.scopus.com/inward/record.url?scp=85125643114&partnerID=8YFLogxK
U2 - https://doi.org/10.1111/jth.15676
DO - https://doi.org/10.1111/jth.15676
M3 - Article
C2 - 35171522
SN - 1538-7933
VL - 20
SP - 1126
EP - 1137
JO - Journal of thrombosis and haemostasis
JF - Journal of thrombosis and haemostasis
IS - 5
ER -