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Joint status of patients with nonsevere hemophilia A

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 pat...

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Autores principales: Zwagemaker, Anne‐Fleur, Kloosterman, Fabienne R., Hemke, Robert, Gouw, Samantha C., Coppens, Michiel, Romano, Lorenzo G. R., Kruip, Marieke J. H. A., Cnossen, Marjon H., Leebeek, Frank W. G., Hutten, Barbara A., Maas, Mario, Fijnvandraat, Karin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9314729/
https://www.ncbi.nlm.nih.gov/pubmed/35171522
http://dx.doi.org/10.1111/jth.15676
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author Zwagemaker, Anne‐Fleur
Kloosterman, Fabienne R.
Hemke, Robert
Gouw, Samantha C.
Coppens, Michiel
Romano, Lorenzo G. R.
Kruip, Marieke J. H. A.
Cnossen, Marjon H.
Leebeek, Frank W. G.
Hutten, Barbara A.
Maas, Mario
Fijnvandraat, Karin
author_facet Zwagemaker, Anne‐Fleur
Kloosterman, Fabienne R.
Hemke, Robert
Gouw, Samantha C.
Coppens, Michiel
Romano, Lorenzo G. R.
Kruip, Marieke J. H. A.
Cnossen, Marjon H.
Leebeek, Frank W. G.
Hutten, Barbara A.
Maas, Mario
Fijnvandraat, Karin
author_sort Zwagemaker, Anne‐Fleur
collection PubMed
description 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.
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spelling pubmed-93147292022-07-30 Joint status of patients with nonsevere hemophilia A Zwagemaker, Anne‐Fleur Kloosterman, Fabienne R. Hemke, Robert Gouw, Samantha C. Coppens, Michiel Romano, Lorenzo G. R. Kruip, Marieke J. H. A. Cnossen, Marjon H. Leebeek, Frank W. G. Hutten, Barbara A. Maas, Mario Fijnvandraat, Karin J Thromb Haemost HAEMOSTASIS 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. John Wiley and Sons Inc. 2022-03-07 2022-05 /pmc/articles/PMC9314729/ /pubmed/35171522 http://dx.doi.org/10.1111/jth.15676 Text en © 2022 The Authors. Journal of Thrombosis and Haemostasis published by Wiley Periodicals LLC on behalf of International Society on Thrombosis and Haemostasis. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ (https://creativecommons.org/licenses/by-nc-nd/4.0/) License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.
spellingShingle HAEMOSTASIS
Zwagemaker, Anne‐Fleur
Kloosterman, Fabienne R.
Hemke, Robert
Gouw, Samantha C.
Coppens, Michiel
Romano, Lorenzo G. R.
Kruip, Marieke J. H. A.
Cnossen, Marjon H.
Leebeek, Frank W. G.
Hutten, Barbara A.
Maas, Mario
Fijnvandraat, Karin
Joint status of patients with nonsevere hemophilia A
title Joint status of patients with nonsevere hemophilia A
title_full Joint status of patients with nonsevere hemophilia A
title_fullStr Joint status of patients with nonsevere hemophilia A
title_full_unstemmed Joint status of patients with nonsevere hemophilia A
title_short Joint status of patients with nonsevere hemophilia A
title_sort joint status of patients with nonsevere hemophilia a
topic HAEMOSTASIS
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9314729/
https://www.ncbi.nlm.nih.gov/pubmed/35171522
http://dx.doi.org/10.1111/jth.15676
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