Article
Hematology
Lars L. F. G. Valke, Marieke J. A. Verhagen, Bart T. P. M. Mulders, Robert Polenewen, Nicole M. A. Blijlevens, Joop H. Jansen, Hassan Mansouritorghabeh, Einas Elsheikh, Birgit M. Reipert, Peter L. Turecek, James S. O'Donnell, Sanna R. Rijpma, Saskia E. M. Schols, Waander L. van Heerde, Danielle Meijer
Summary: The NusBA is a modified version of the NBA assay that can detect very low-titer inhibitors. It has added value in early inhibitor detection and therapy adjustments in patients with congenital HA and AHA.
THROMBOSIS RESEARCH
(2023)
Article
Hematology
Jens Mueller, Sonja Neimanis, Joerg Kahle, Thilo Albert, Stephan Schultze Strasser, Bonita Rup, Bernd Poetzsch, Christoph Koenigs, Johannes Oldenburg, ABIRISK Consortium
Summary: This study comprehensively validated different anti-FVIII-antibody assays in two laboratories, providing novel insights into the impact of pre-analytical sample treatment and the comparability of test results generated by methodically different assays.
Article
Medicine, General & Internal
Bipin P. Kulkarni, Kirti Ghargi, Chandrakala Shanmukhaiah, Shrimati D. Shetty
Summary: The objective of this study is to standardize the Bethesda assay for the VWF inhibitors and to estimate the VWD inhibitor titer in two cases of congenital type 3 VWD, which developed the VWF inhibitors. The study successfully standardized the Bethesda assay for the quantitation of VWF inhibitors in two patients with congenital type 3 VWD with inhibitors.
FRONTIERS IN MEDICINE
(2022)
Article
Hematology
Albain Chansavang, Aurelien Philippe, Ines Bozinovic, Khalil Ben Hadj Ali, David Smadja, Dominique Helley, Luc Darnige, Laetitia Mauge
Summary: This study evaluated the performance of a commercial ELISA method for detecting anti-FVIII IgG in patients with acquired hemophilia A. The results showed 100% sensitivity and specificity, and correlated with the traditional Bethesda assay. These data suggest the potential use of this method for both diagnosis and follow-up of AHA patients.
ANNALS OF HEMATOLOGY
(2022)
Article
Hematology
Behnaz Pezeshkpoor, Nadja Sereda, Ann-Cristin Berkemeier, Isabell Matuschek, Nadine Schwarz, Peter L. Turecek, Silvia Horneff, Claudia Klein, Georg Goldmann, Natascha Marquardt, Thilo Albert, Jens Mueller, Johannes Oldenburg
Summary: Two patients with mild hemophilia A developed antibodies against the PEG moiety of the therapeutic 40PEG-BDDFVIII. The antibodies neutralized the efficacy of PEGylated therapeutics.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2023)
Article
Medicine, General & Internal
Nikolas Gutierrez, James Park, Terrance Leighton
Summary: DOACs are commonly used anticoagulants for conditions like atrial fibrillation, but can also affect other factors in rare cases. The variable effects of DOACs on PT and PTT can present challenges in detecting bleeding disorders, prompting consideration for expedited workup in patients with significant abnormalities.
CUREUS JOURNAL OF MEDICAL SCIENCE
(2021)
Article
Medical Laboratory Technology
Armando Tripodi, Veena Chantarangkul, Cristina Novembrino, Erica Scalambrino, Massimo Boscolo-Anzoletti, Marigrazia Clerici, Federica Rossi, Flora Peyvandi
Summary: This study demonstrates that by making slight modifications to the OSA for FVIII, it can reliably measure the concentration of emicizumab and assist clinicians in managing patients on emicizumab. Additionally, the use of CA assay with bovine reagents can effectively measure the inhibitors to FVIII in patients receiving emicizumab treatment.
CLINICAL CHEMISTRY AND LABORATORY MEDICINE
(2021)
Review
Hematology
Piet Meijer, Flora Peyvandi, Guy Young, Rajiv Pruthi, Silmara de Lima Montalvao, Steve Kitchen
Summary: This guidance document provides recommendations on the measurement of factor VIII (FVIII) and factor IX (FIX) inhibitors, including screening, assay principle, sample and testing requirements, interpretation, quality assurance, interferences, and recent developments. It focuses on standardizing the laboratory measurement of FVIII and FIX type I inhibitors based on published data and expert opinion.
INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY
(2023)
Article
Hematology
Arnaud Bonnefoy, Clemence Merlen, Evemie Dube, Hadrien Claus-Desbonnet, Georges-Etienne Rivard, Jean St-Louis
Summary: The Ig patterns of AHA patients at diagnosis are varied and may be associated with underlying conditions. The presence of IgG1, IgG4, and IgM may have differential predictive significance for bleeding severity and outcomes, with early determination of Ig profile potentially helping to identify high-risk patients.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2021)
Review
Hematology
Kenneth C. Childers, Shaun C. Peters, Paul Clint Spiegel
Summary: Advances in structural studies of blood coagulation factor VIII (FVIII) have provided important insights into the biochemical properties of FVIII, particularly the mutations associated with hemophilia A. By analyzing the atomic details of FVIII structures, researchers can design recombinant FVIII with improved circulatory half-life. Recent structural studies of FVIII bound to inhibitory antibodies have also enhanced our understanding of FVIII binding to activated platelet membranes and the formation of the intrinsic tenase complex.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2022)
Article
Chemistry, Analytical
Ming Xu, Jiajing Zhou, Yong Cheng, Zhicheng Jin, Alex E. Clark, Tengyu He, Wonjun Yim, Yi Li, Yu-Ci Chang, Zhuohong Wu, Pavla Fajtova, Anthony J. O'Donoghue, Aaron F. Carlin, Michael D. Todd, Jesse Jokerst
Summary: A novel self-immolative fluorescent probe (MP590) has been developed for the highly selective and sensitive detection of SARS-CoV-2 main protease (M-pro). This probe can measure and monitor M-pro activity and quantitatively evaluate M-pro inhibition in infected cells.
ANALYTICAL CHEMISTRY
(2022)
Article
Hematology
Amber Vander Kooi, Shuaishuai Wang, Meng-Ni Fan, Alex Chen, Junping Zhang, Chun-Yu Chen, Xiaohe Cai, Barbara A. Konkle, Weidong Xiao, Lei Li, Carol H. Miao
Summary: The N-glycosylation of FVIII has a significant impact on its immunogenicity, as shown in this study. Gene mutations were introduced to eliminate glycosylation at potential sites, and it was found that FVIII activity remained stable while immune responses varied. A specific glycopeptide epitope surrounding one of the glycosylation sites was identified and characterized for its activation of T cells.
Article
Plant Sciences
Muhammad Jamil, Jian You Wang, Djibril Yonli, Tsuyoshi Ota, Lamis Berqdar, Hamidou Traore, Ouedraogo Margueritte, Binne Zwanenburg, Tadao Asami, Salim Al-Babili
Summary: This study evaluated the efficacy of three potent SL analogs in laboratory, greenhouse, and field conditions for reducing the germination of Striga seeds. The results showed that these analogs have promising potential as simple suicidal agents to combat Striga infestation.
Article
Hematology
Debadrita Ray, Narender Kumar, Chander Hans, Anita Kler, Richa Jain, Deepak Bansal, Amita Trehan, Arihant Jain, Pankaj Malhotra, Jasmina Ahluwalia
Summary: This study assessed the prevalence and clinicopathological correlates of inhibitors in a cohort of Indian patients with hemophilia A. Inhibitors were detected in 35 out of 592 patients (5.9%). These patients may have a higher risk of intracranial bleeding.
INDIAN JOURNAL OF HEMATOLOGY AND BLOOD TRANSFUSION
(2022)
Article
Immunology
Estelle K. Ronayne, Shaun C. Peters, Joseph S. Gish, Celena Wilson, H. Trent Spencer, Christopher B. Doering, Pete Lollar, P. Clint Spiegel, Kenneth C. Childers
Summary: This study reports the X-ray crystal structure of a B domain-deleted bioengineered fVIII bound to the non-classical antibody inhibitor, G99. While binding to G99 does not disrupt the overall domain architecture of fVIII, the C2 domain undergoes an approximately 8 angstrom translocation along with breaking multiple domain-domain interactions. Analysis of normalized B-factor values reveals a decrease in thermal motion in certain solvent-exposed loops in the presence of inhibitory antibodies, providing insights into the structural dynamics of binding non-classical inhibitors and the cooperative nature between anti-C1 and anti-C2 domain inhibitors.
FRONTIERS IN IMMUNOLOGY
(2021)