Tachyphylaxis and reproducibility of desmopressin response in perioperative persons with nonsevere hemophilia A: implications for clinical practice
CONCLUSION: Tachyphylaxis in the surgical setting was considerably more pronounced than previously reported, with FVIII:C at D1 and D2 of 36% to 43% of the initial response. Our results may have important implications for monitoring repeated desmopressin treatment when used perioperatively.PMID:38660455 | PMC:PMC11039391 | DOI:10.1016/j.rpth.2024.102367 (Source: Thrombosis and Haemostasis)
Source: Thrombosis and Haemostasis - April 25, 2024 Category: Hematology Authors: L G R Romano L M Sch ütte R M van Hest K Meijer B A P Laros-van Gorkom L Nieuwenhuizen J Eikenboom F C J I Heubel-Moenen N Uitslager M Coppens K Fijnvandraat M H E Driessens S Polinder M H Cnossen F W G Leebeek R A A Math ôt M J H A Kruip DAVID and SYMP Source Type: research
Navigating challenges in diagnosing acquired hemophilia A: A case report from Syria
Early diagnosis and intervention are crucial for better outcomes in acquired hemophilia A. Key clinical messageAcquired hemophilia A is a rare bleeding disorder. Rapid diagnosis with prolonged aPTT and low FVIII, and immediate use of bypassing agents and steroids are crucial for better outcomes, highlighting the importance of early recognition and management. (Source: Clinical Case Reports)
Source: Clinical Case Reports - April 25, 2024 Category: General Medicine Authors: Oubai Nayouf,
Miriam Laflouf,
Ibrahim Hamdan,
Ibrahim Alghazawi,
Omar Aldairi,
Ameen Sulaiman Tags: CASE REPORT Source Type: research
Incidence of deep venous thrombosis in patients with hemophilia undergoing bilateral simultaneous total knee arthroplasty: a retrospective cohort study
Hemophilic arthropathy usually affects the knees bilaterally. In order to reduce costs and improve rehabilitation, bilateral simultaneous total knee arthroplasty (TKA) can be performed. However, pharmacologica... (Source: BMC Musculoskeletal Disorders)
Source: BMC Musculoskeletal Disorders - April 24, 2024 Category: Orthopaedics Authors: Qian Zhang, Lingying Zhao, Nicoletta Riva, Ziqiang Yu, Miao Jiang, Alexander Gatt and Jiong Jiong Guo Tags: Research Source Type: research
Cost-Effectiveness of Recombinant Factor IX Fc Prophylaxis and Recombinant Factor IX On-Demand Treatment in Patients with Haemophilia B Without Inhibitors
CONCLUSIONS: rFIXFc prophylaxis provides improved health outcomes and lower costs, and represents a cost-effective treatment option compared with rFIX on-demand for adolescent and adult male patients with haemophilia B. This comparative assessment of cost-effectiveness should help to inform both clinicians and healthcare policy makers when making treatment decisions for patients with haemophilia B.PMID:38652439 | DOI:10.1007/s12325-024-02841-w (Source: Adv Data)
Source: Adv Data - April 23, 2024 Category: Epidemiology Authors: Michal Pochopien Anna Tytu ła Mondher Toumi Aletta Falk Nicoletta Martone Zalmai Hakimi Daniel Eriksson Source Type: research
Cost-Effectiveness of Recombinant Factor IX Fc Prophylaxis and Recombinant Factor IX On-Demand Treatment in Patients with Haemophilia B Without Inhibitors
CONCLUSIONS: rFIXFc prophylaxis provides improved health outcomes and lower costs, and represents a cost-effective treatment option compared with rFIX on-demand for adolescent and adult male patients with haemophilia B. This comparative assessment of cost-effectiveness should help to inform both clinicians and healthcare policy makers when making treatment decisions for patients with haemophilia B.PMID:38652439 | DOI:10.1007/s12325-024-02841-w (Source: Adv Data)
Source: Adv Data - April 23, 2024 Category: Epidemiology Authors: Michal Pochopien Anna Tytu ła Mondher Toumi Aletta Falk Nicoletta Martone Zalmai Hakimi Daniel Eriksson Source Type: research
Cost-Effectiveness of Recombinant Factor IX Fc Prophylaxis and Recombinant Factor IX On-Demand Treatment in Patients with Haemophilia B Without Inhibitors
ConclusionsrFIXFc prophylaxis provides improved health outcomes and lower costs, and represents a cost-effective treatment option compared with rFIX on-demand for adolescent and adult male patients with haemophilia B. This comparative assessment of cost-effectiveness should help to inform both clinicians and healthcare policy makers when making treatment decisions for patients with haemophilia B. (Source: Advances in Therapy)
Source: Advances in Therapy - April 23, 2024 Category: Drugs & Pharmacology Source Type: research
Abnormal frequency of the memory B cell subsets and plasmablasts in patients with congenital severe hemophilia A: correlation with "Inhibitor" formation
CONCLUSION: This study is the first to demonstrate a dysregulated proportion of CD19+IgD+CD27+ non-class-switched memory B cells and CD19+CD27hiCD38hi plasmablasts in patients with severe HA. Therefore, strategies targeting memory B-cell/plasmablast differentiation may have promising outcomes in the management of inhibitor formation in patients with severe HA.PMID:38625415 | DOI:10.1007/s44313-024-00017-7 (Source: Blood Research)
Source: Blood Research - April 16, 2024 Category: Hematology Authors: Omid Reza Zekavat Yasaman Movahednezhad Amin Shahsavani Sezaneh Haghpanah Negin Shokrgozar Hossein Golmoghaddam Mehdi Kalani Mohammad Reza Bordbar Nargess Arandi Source Type: research
The Role of Clot Waveform Analysis and Related Parameters in the Diagnosis and Treatment of Hemophilia A
CONCLUSION: CWA is an economical and practical tool, and its related parameters are associated with the severity of HA. It has promising clinical prospects in predicting FVIII:C levels and individualized treatment when HA patients undergo replacement therapy.PMID:38626899 | DOI:10.1055/s-0044-1786029 (Source: Thrombosis and Haemostasis)
Source: Thrombosis and Haemostasis - April 16, 2024 Category: Hematology Authors: Guanghao Song Yangbin Wang Lilei Zhang Mao Xia Source Type: research
Abnormal frequency of the memory B cell subsets and plasmablasts in patients with congenital severe hemophilia A: correlation with "Inhibitor" formation
CONCLUSION: This study is the first to demonstrate a dysregulated proportion of CD19+IgD+CD27+ non-class-switched memory B cells and CD19+CD27hiCD38hi plasmablasts in patients with severe HA. Therefore, strategies targeting memory B-cell/plasmablast differentiation may have promising outcomes in the management of inhibitor formation in patients with severe HA.PMID:38625415 | PMC:PMC11021380 | DOI:10.1007/s44313-024-00017-7 (Source: Blood Research)
Source: Blood Research - April 16, 2024 Category: Hematology Authors: Omid Reza Zekavat Yasaman Movahednezhad Amin Shahsavani Sezaneh Haghpanah Negin Shokrgozar Hossein Golmoghaddam Mehdi Kalani Mohammad Reza Bordbar Nargess Arandi Source Type: research
Comparative Effectiveness of Valoctocogene Roxaparvovec and Prophylactic Factor VIII Replacement in Severe Hemophilia A
ConclusionsPS-adjusted analyses were consistent with prior intra-individual comparisons. Compared with participants receiving prophylactic FVIII, the participants receiving valoctocogene roxaparvovec experienced lower ABR, and a higher proportion had zero bleeds.Trail RegistrationClinicalTrials.gov identifier, NCT03370913. (Source: Advances in Therapy)
Source: Advances in Therapy - April 15, 2024 Category: Drugs & Pharmacology Source Type: research
Three-year outcomes of valoctocogene roxaparvovec gene therapy for hemophilia A
CONCLUSIONS: Hemostatic efficacy was maintained, and safety remained unchanged from previous years.PMID:38614387 | DOI:10.1016/j.jtha.2024.04.001 (Source: Thrombosis and Haemostasis)
Source: Thrombosis and Haemostasis - April 13, 2024 Category: Hematology Authors: Bella Madan Margareth C Ozelo Priyanka Raheja Emily Symington Doris V Quon Andrew D Leavitt Steven W Pipe Gillian Lowe Gili Kenet Mark T Reding Jane Mason Michael Wang Annette von Drygalski Robert Klamroth Susan Shapiro Herv é Chambost Amy L Dunn Johanne Source Type: research
Three-year outcomes of valoctocogene roxaparvovec gene therapy for hemophilia A
CONCLUSIONS: Hemostatic efficacy was maintained, and safety remained unchanged from previous years.PMID:38614387 | DOI:10.1016/j.jtha.2024.04.001 (Source: Thrombosis and Haemostasis)
Source: Thrombosis and Haemostasis - April 13, 2024 Category: Hematology Authors: Bella Madan Margareth C Ozelo Priyanka Raheja Emily Symington Doris V Quon Andrew D Leavitt Steven W Pipe Gillian Lowe Gili Kenet Mark T Reding Jane Mason Michael Wang Annette von Drygalski Robert Klamroth Susan Shapiro Herv é Chambost Amy L Dunn Johanne Source Type: research
Concomitant large deletion and de novo duplication of factor VIII gene in an Indian patient with severe Hemophilia A
(Source: Annals of Hematology)
Source: Annals of Hematology - April 12, 2024 Category: Hematology Source Type: research
