Background: Patients with hemophilia A can develop joint hemarthroses, degenerative changes, and eventually undergo total shoulder arthroplasty (TSA). Few data exist concerning complications and prosthesis survival after TSA in this population.
Questions/purposes: (1) Is hemophilia A associated with more bleeding and thromboembolic adverse events after TSA relative to matched controls? (2) Is 5-year TSA prosthesis survival reduced in patients with hemophilia A compared with matched controls?
Methods: The 2010 to 2022 PearlDiver M161 database was used to identify patients who underwent primary anatomic or reverse TSA. Given that the X-linked recessive condition hemophilia A presents nearly exclusively in males, male patients with hemophilia A who underwent TSA were matched 1:10 with male patients without hemophilia who underwent TSA based on age and Elixhauser comorbidity index (ECI). This yielded 73 patients with hemophilia A who underwent TSA who were matched 1:10 with 729 patients without hemophilia. Ninety-day adverse events were compared with multivariable analysis. Revision within 5 years was assessed using Kaplan-Meier analysis.
Results: Compared with the control cohort, patients with hemophilia had greater odds of bleeding issues (hematoma, OR 6.8 [95% CI 3.0 to 15.3]; p < 0.001; anemia, OR 2.5 [95% CI 1.5 to 4.2]; p < 0.001, transfusion, OR 5.0 [95% CI 2.4 to 10.3]; p < 0.001), venous thromboembolic events (VTE) (OR 1.9 [95% CI 1.1 to 3.1]; p = 0.01), and prosthetic loosening (OR 3.5 [95% CI 1.4 to 8.0]; p = 0.004). Based on available data, 5-year implant survival was not different in patients with hemophilia (97.3% [95% CI 93.6% to 100.0%]) relative to matched controls (95.2% [95% CI 93.4% to 97.2%]; p = 0.60).
Conclusion: The elevated risks of both 90-day bleeding complications (hematoma, anemia, and transfusion) and VTE (DVT and PE) in patients with hemophilia emphasize the special challenges of carefully balancing factor replacement and VTE prophylaxis pre-, intra-, and postoperatively on an individual patient basis with careful hematologist coordination. Further study on Factor VIII levels and targets as well as tranexamic acid and VTE prophylaxis in this population is necessary to provide further guidance. Furthermore, 5-year implant survival was not different between patients with hemophilia and matched controls (patients without hemophilia) based on available data, suggesting that TSA survivorship remains durable and may be offered to patients in this population as indicated.
Level Of Evidence: Level III, therapeutic study.
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http://dx.doi.org/10.1097/CORR.0000000000003209 | DOI Listing |
Life (Basel)
November 2024
Department of Biomedical and Clinical Sciences, Università degli Studi di Milano, 20157 Milan, Italy.
Background: Although more than four years have passed since the pandemic began, SARS-CoV-2 continues to be of concern. Therefore, research into the underlying mechanisms that contribute to the development of the disease, especially in more severe forms, remains a priority. Sustained activation of the complement (CS), contact (CAS), and fibrinolytic and kinin-kallikrein systems (KKS) has been shown to play a central role in the pathogenesis of the disease.
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January 2025
Anesthesiology and Perioperative Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA.
Clinical Features: Sickle cell patients may develop a multitude of antibodies and experience life-threatening events with transfusion such as hyperhemolysis syndrome or delayed hemolytic transfusion reaction. Further transfusion may not be possible in such cases.
Therapeutic Challenge: When conventional blood products are not available for transfusion yet the patient requires additional oxygen-carrying support, artificial oxygen carriers may be required.
Exp Mol Med
January 2025
Department of Physiology, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea.
Hemophilia A (HA) is caused by mutations in coagulation factor VIII (FVIII). Genome editing in conjunction with patient-derived induced pluripotent stem cells (iPSCs) is a promising cell therapy strategy, as it replaces dysfunctional proteins resulting from genetic mutations with normal proteins. However, the low expression level and short half-life of FVIII still remain significant limiting factors in the efficacy of these approaches in HA.
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December 2024
Research Department of Haematology, UCL Cancer Institute, University College London, London, United Kingdom.
Various therapeutic strategies have been developed to treat Pancreatic Cancer (PaCa). Unfortunately, most efforts have proved unfruitful, as the poor prognosis observed in this disease has only attained little improvement in the past 40 years. Recently, deeper understanding of the immune system and its interaction with malignant tumors have allowed significant advances in immunotherapy.
View Article and Find Full Text PDFBr J Haematol
January 2025
Hematology Department, Beijing Children's Hospital, Hemophilia Comprehensive Care Center, National Center for Children's Health, Capital Medical University, Beijing, China.
Avatrombopag is a newly approved thrombopoietin receptor agonist for second-line treatment of chronic immune thrombocytopenia (ITP) in adults. Our previous study showed its efficacy and safety in a small sample of paediatric ITP patients. However, large samples and long-term data are still lacking.
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