Article
Immunology
Cecile L. Karsenty, Susan E. Kirk, Hannah L. Helber, Jose M. Esquilin, Jenny M. Despotovic, Amanda B. Grimes
Summary: This article reviews thrombotic thrombocytopenic purpura (TTP) and discusses two cases of TTP in adolescents, emphasizing the need to consider late-onset congenital TTP (cTTP). The importance of early confirmation of ADAMTS13 enzyme deficiency and the presence or absence of ADAMTS13 inhibitor is demonstrated in order to initiate appropriate life-saving therapies. Ultimately, molecular testing is crucial for distinguishing between congenital and acquired immune-mediated TTP.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Hematology
Jana van den Berg, Johanna A. Kremer Hovinga, Claudia Pfleger, Inga Hegemann, Gregor Stehle, Andreas Holbro, Jan-Dirk Studt
Summary: Immune thrombotic thrombocytopenic purpura (iTTP) is a life-threatening disease that can be treated with the anti-CD38 antibody daratumumab to eradicate persistent ADAMTS13 inhibitory autoantibodies and restore normal ADAMTS13 activity.
Article
Hematology
James N. George
Summary: The article narrates the author's personal experience with TTP, tracing the history and development of treatment methods. Through collaborative research and patient data, a deeper understanding of TTP diagnosis and treatment, as well as the differences between hTTP and iTTP, was gained.
SEMINARS IN THROMBOSIS AND HEMOSTASIS
(2022)
Article
Physiology
Suhan Zhou, Jie Guo, Xinxin Liao, Qin Zhou, Xingyu Qiu, Shan Jiang, Nan Xu, Xiaohua Wang, Liang Zhao, Weipeng Hu, Lanyu Xie, Peng Xie, Yu Cui, Yi Yang, Andreas Patzak, Pontus B. Persson, Jianhua Mao, En Yin Lai
Summary: ADAMTS13 shows promise as a new treatment option for acute kidney injury, as it reduces oxidative stress and inflammation in renal ischemia/reperfusion injury.
Review
Hematology
Konstantine Halkidis, X. Long Zheng
Summary: ADAMTS13 is a crucial plasma enzyme for normal hemostasis, and deficiency caused by acquired autoantibodies can lead to a potentially fatal blood disorder. Recent research has shown that ADAMTS13 exists in multiple conformations and can be regulated by antibodies in different ways.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2022)
Article
Hematology
Emmanuel J. Favaloro, Soma Mohammed, Kent Chapman, Priscilla Swanepoel, Diane Zebeljan, Opelo Sefhore, Erica Malan, Joanne Clifford, Agnes Yuen, Dea Donikian, Mayuko Kondo, Elizabeth Duncan, Sunil Abraham, Joanne Beggs, Ritesh Chatrapati, Joanne Perel, Robyn Coleman, Nathan Klose, Danny Hsu, Penelope Motum, Chee Wee Tan, Timothy Brighton, Leonardo Pasalic
Summary: The study evaluated the utility of a new automated test for ADAMTS13 activity, which showed similar results to the comparator ELISA with slight bias. Detection of ADAMTS13 inhibitors was also comparable with slight bias. The HemosIL AcuStar ADAMTS13 Activity assay provides a fast and accurate means for quantitating plasma levels of ADAMTS13, aiding in the identification/exclusion of TTP/ADAMTS13 and potentially other applications.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2021)
Review
Medical Laboratory Technology
Konstantinos Dimopoulos, Armando Tripodi, Jens P. Goetze
Summary: Thrombotic thrombocytopenic purpura (TTP) is a rare and potentially fatal disease. Rapid diagnosis is crucial for patient outcomes, and measurement of ADAMTS13 enzyme activity can establish the diagnosis of TTP with high accuracy. Besides clinical history, appropriate laboratory assessment is essential for diagnosis and treatment of suspected TTP cases.
CRITICAL REVIEWS IN CLINICAL LABORATORY SCIENCES
(2023)
Article
Hematology
Dearbhla Doherty, Michelle Lavin, Mary Byrne, Margaret Nolan, Jamie M. O'Sullivan, Kevin Ryan, Niamh M. O'Connell, Sandra L. Haberichter, Pamela A. Christopherson, Jorge Di Paola, Paula D. James, James S. O'Donnell
Summary: This study investigates the role of enhanced von Willebrand factor (VWF) clearance in patients with mild to moderate reductions in plasma VWF:Ag (low VWF). The results show that 20% of the low-VWF cohort demonstrate significantly enhanced VWF clearance, which does not affect the VWF propeptide (VWFpp)/VWF antigen (VWF:Ag) ratio. The discrepancy between the VWFpp/VWF:Ag ratio and desmopressin fall-off rates in patients with mild quantitative VWD may reflect alterations in VWFpp clearance kinetics. Furthermore, patients with enhanced VWF clearance have significantly lower bleeding scores compared to those with reduced VWF biosynthesis.
Article
Hematology
Steven de Maat, Chantal C. Clark, Arjan D. Barendrecht, Simone Smits, Nadine D. van Kleef, Hinde El Otmani, Manon Waning, Marc van Moorsel, Michael Szardenings, Nicolas Delaroque, Kristof Vercruysse, Rolf T. Urbanus, Silvie Sebastian, Peter J. Lenting, Christoph Hagemeyer, Thomas Renne, Karen Vanhoorelbeke, Claudia Tersteeg, Coen Maas
Summary: Thrombotic microangiopathies are characterized by attacks of disseminated microvascular thrombosis. This study developed a fusion protein called Microlyse that can target and activate plasminogen on microthrombi, showing greater efficacy for the treatment of TTP compared to current methods.
Article
Medicine, General & Internal
Ralph Wendt, Sven Kalbitz, Felix Otto, Tanja Falter, Joachim Beige, Heidi Rossmann, Bernhard Lammle
Summary: A 43-year-old Armenian patient was diagnosed with salmonella infection and thrombotic microangiopathy, which resolved after antibiotic treatment. Further diagnosis revealed congenital thrombotic thrombocytopenic purpura, based on evidence of deficient ADAMTS13 activity and a rare complex ADAMTS13 allele. Plasma infusion was used to calculate the half-life of ADAMTS13 and investigate suspected smoldering TTP activity.
FRONTIERS IN MEDICINE
(2021)
Article
Hematology
Claire Kizlik-Masson, Ivan Peyron, Stephane Gangnard, Gaelle Le Goff, Solen M. Lenoir, Sandra Damodaran, Marie Clavel, Stephanie Roullet, Veronique Regnault, Antoine Rauch, Flavien Vincent, Emmanuelle Jeanpierre, Annabelle Dupont, Catherine Ternisien, Thibault Donnet, Olivier D. Christophe, Eric van Belle, Cecile Denis, Caterina Casari, Sophie Susen, Peter J. Lenting
Summary: A nanobody (KB-VWF-D3.1) is identified to distinguish proteolyzed and non-proteolyzed VWF and monitor VWF degradation levels. Reduced intact-VWF levels are observed in patients with von Willebrand disease, aortic stenosis, and patients receiving mechanical circulatory support.
Article
Hematology
Valeria Sutana Ladeira, Amanda Rodrigues Barbosa, Marina Mendes Oliveira, Leticia Goncalves Resende Ferreira, Wander Valadares de Oliveira Junior, Cristiane de Oliveira Reno, Edna Afonso Reis, Daniel Goncalves Chaves, Luci Maria Sant'Ana Dusse, Herica Lima dos Santos, Melina de Barros Pinheiro, Danyelle Romana Alves Rios
Summary: This study found that the ADAMTS13-VWF axis is altered in SCD patients compared to healthy individuals, but there is no association between these markers and the use of hydroxyurea (HU).
ANNALS OF HEMATOLOGY
(2021)
Article
Hematology
Jiangguo Lin, Xiaoru Ding, Pu Yang, Silu Liu, Quhuan Li, Miguel A. Cruz, Jing-fei Dong, Ying Fang, Jianhua Wu
Summary: Platelets need to be recruited quickly to the site of vascular injury for hemostasis, which is regulated by the adhesive ligand von Willebrand factor (VWF) multimers. ADAMTS13, a metalloproteinase, reduces the size of VWF and its proteolytic kinetics have been studied. However, the mechanism of how ADAMTS13 cleaves VWF in flowing blood is still unclear.
THROMBOSIS RESEARCH
(2023)
Article
Hematology
Masanori Matsumoto, Yoshitaka Miyakawa, Koichi Kokame, Yasunori Ueda, Hideo Wada, Satoshi Higasa, Hideo Yagi, Yoshiyuki Ogawa, Kazuya Sakai, Toshiyuki Miyata, Eriko Morishita, Yoshihiro Fujimura
Summary: Thrombotic thrombocytopenic purpura (TTP) is a life-threatening condition that requires appropriate diagnosis and treatment. Clinical findings such as thrombocytopenia and hemolytic anemia, along with reduced activity of ADAMTS13 below 10%, are used to diagnose TTP. Treatment methods for TTP include FFP transfusion, plasma exchange, corticosteroids, and antibody therapy.
INTERNATIONAL JOURNAL OF HEMATOLOGY
(2023)
Article
Biotechnology & Applied Microbiology
Brady Trevisan, Alshaimaa Morsi, Julio Aleman, Martin Rodriguez, Jordan Shields, Diane Meares, Andrew M. Farland, Christopher B. Doering, H. Trent Spencer, Anthony Atala, Aleks Skardal, Christopher D. Porada, Graca Almeida-Porada
Summary: The study utilized microfluidic technology to simulate physiological shear stress in liver sinusoids and investigate the effects of shear stress on an innovative biological approach for treating Hemophilia A. The research revealed novel pathways through which endogenous FVIII and vWF are affected by shear stress.
FRONTIERS IN BIOENGINEERING AND BIOTECHNOLOGY
(2021)
Article
Multidisciplinary Sciences
Kathrin Rack, Volker Huck, Masoud Hoore, Dmitry A. Fedosov, Stefan W. Schneider, Gerhard Gompper
SCIENTIFIC REPORTS
(2017)
Article
Dermatology
S. Springer, M. Zieger, U. C. Hipler, J. Lademann, V Albrech, R. Bueckle, C. Mess, M. Kaatz, V Huck
SKIN RESEARCH AND TECHNOLOGY
(2019)
Article
Cell & Tissue Engineering
P. Netsch, S. Elvers-Hornung, S. Uhlig, H. Klueter, V. Huck, F. Kirschhoefer, G. Brenner-Weiss, K. Janetzko, H. Solz, P. Wuchter, P. Bugert, K. Bieback
STEM CELL RESEARCH & THERAPY
(2018)
Article
Multidisciplinary Sciences
Jan P. Nicolay, Verena Thorn, Christoph Daniel, Kerstin Amann, Balasaheb Siraskar, Florian Lang, Carina Hillgruber, Tobias Goerge, Stefan Hoffmann, Christian Gorzelanny, Volker Huck, Christian Mess, Tobias Obser, Reinhard Schneppenheim, Ingrid Fleming, Matthias F. Schneider, Stefan W. Schneider
SCIENTIFIC REPORTS
(2018)
Article
Hematology
Reinhard Schneppenheim, Natalie Hellermann, Maria A. Brehm, Ulrike Klemm, Tobias Obser, Volker Huck, Stefan W. Schneider, Cecile V. Denis, Alexander Tischer, Matthew Auton, Winfried Maerz, Emma-Ruoqi Xu, Matthias Wilmanns, Rainer B. Zotz
Editorial Material
Hematology
Kandace Gollomp, Mortimer Poncz
Article
Biochemistry & Molecular Biology
Angelica Sandoval-Perez, Ricarda M. L. Berger, Adiran Garaizar, Stephen E. Farr, Maria A. Brehm, Gesa Koenig, Stefan W. Schneider, Rosana Collepardo-Guevara, Volker Huck, Joachim O. Raedler, Camilo Aponte-Santamaria
NUCLEIC ACIDS RESEARCH
(2020)
Review
Pharmacology & Pharmacy
Christian Gorzelanny, Christian Mess, Stefan W. Schneider, Volker Huck, Johanna M. Brandner
Article
Cell Biology
Matthias F. Schneider, Mohammad A. Fallah, Christian Mess, Tobias Obser, Reinhard Schneppenheim, Alfredo Alexander-Katz, Stefan W. Schneider, Volker Huck
BMC MOLECULAR AND CELL BIOLOGY
(2020)
Article
Dermatology
Lynhda Nguyen, Christian Mess, Stefan W. Schneider, Volker Huck, Katharina Herberger
Summary: With the increasing popularity of tattoos, there is a greater awareness of dermatological complications associated with tattoos. This study introduces a non-invasive diagnostic technique called multiphoton tomography with fluorescence lifetime imaging to examine the morphological and metabolic status of tattooed human skin. It allows for the visualization of tattoo pigments and analysis of skin changes after tattoo ink injection.
EXPERIMENTAL DERMATOLOGY
(2022)
Article
Dermatology
Lynhda Nguyen, Christian Mess, Stefan W. Schneider, Volker Huck, Katharina Herberger
Summary: Laser-assisted drug delivery (LADD) is an effective method to enhance the penetration of hyaluronic acid (HA) into the skin, improving hydration and skin rejuvenation. Multiphoton tomography equipped with fluorescence lifetime imaging (MPT-FLIM) is a suitable diagnostic tool for tracking drug delivery and monitoring treatment response.
EXPERIMENTAL DERMATOLOGY
(2023)
Meeting Abstract
Hematology
Reinhard Schneppenheim, Natalie Hellermann, Maria Alexandra Brehm, Ulrike Klemm, Tobias Obser, Volker Huck, Stefan W. Schneider, Cecile V. Denis, Alexander Tischer, Matthew Auton, Winfried Maerz, Emma-Ruoqi Xu, Matthias Wilmanns, Rainer B. Zotz
Meeting Abstract
Dermatology
J. P. Nicolay, V. Thorn, C. Daniel, K. Amann, B. Siraskar, F. Lang, C. Hillgruber, T. Goerge, C. Gorzelanny, V. Huck, T. Obser, R. Schneppenheim, I. Fleming, M. F. Schneider, S. W. Schneider
EXPERIMENTAL DERMATOLOGY
(2018)
Proceedings Paper
Microscopy
Christian Mess, Katharina Zens, Christian Gorzelanny, Dieter Metze, Thomas A. Luger, Karsten Koenig, Stefan W. Schneider, Volker Huck
MULTIPHOTON MICROSCOPY IN THE BIOMEDICAL SCIENCES XVII
(2017)
Meeting Abstract
Dermatology
F. Valentin, C. Mess, I. Hausser, V. Huck, V. Oji
EXPERIMENTAL DERMATOLOGY
(2016)