Results 1 to 10 of about 10,178 (166)

Unbiased plasma proteomics reveals hemolysis during ADAMTS13 relapse in patients with immune TTP [PDF]

Blood Advances
: Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy that is caused by a deficiency of a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13 (ADAMTS13).
Tim Postmus, Maryam O. Subhan, Eva R. Smit, Jan Voorberg, Mari Thomas, Maartje van den Biggelaar, Marie Scully, Rens de Groot, Pieter Langerhorst   +8 more
doaj   +2 more sources

Long Term Follow Up of Hereditary Thrombotic Thrombocytopenic Purpura on Plasma Therapy for 9 Years: A Case Report. [PDF]

Clin Case Rep
ABSTRACT A 7.6‐year‐old boy with hTTP had fever‐induced hemorrhagic rash, MAHA, cerebral infarction, and renal impairment. After plasma therapy, symptoms were partially relieved; 9‐year follow‐up showed regular plasma transfusion was needed, with CKD Stage 3.
Xiaomei D, Ruyue C, Shuang W, Hanyun T, Qiang L, Xiaozhong L.   +5 more
europepmc   +2 more sources

Delayed ADAMTS13 normalization in caplacizumab/PEX–treated patients associated with anti-ADAMTS13 IgG antibody boosting [PDF]

Blood Advances
: Delayed ADAMTS13 normalization was previously reported in a subgroup of caplacizumab/plasma exchange (PEX) cases. To investigate this, anti-ADAMTS13 immunoglobulin G (IgG) levels were analysed at multiple timepointsduring an acute thrombotic ...
Nithya Prasannan, Arshia Latif, John-Paul Westwood, Mari Thomas, Deepak Singh, Marie Scully   +5 more
doaj   +2 more sources

The vWF/ADAMTS13 Ratio as a Potential Marker of Endotheliopathy in Malignancies [PDF]

Biomedicines
Background: Endothelial activation and thromboinflammation play an important role in cancer-associated thrombosis. An imbalance between von Willebrand factor (vWF) and its physiological regulator ADAMTS13 may reflect the development of a prothrombotic ...
Alexander Vorobev, Victoria Bitsadze, Jamilya Khizroeva, Antonina Solopova, Maria Tretyakova, Nilufar Gashimova, Kristina Grigoreva, Irina Kalashnikova, Natalia Makatsariya, Vlada Rubashkina, Yana Sulina, Aidanhanum Iskenderova, Alexandra Antonova, Dmitry Utkin, Jean-Christophe Gris, Ismail Elalamy, Grigoris Gerotziafas, Alexander Makatsariya   +17 more
doaj   +2 more sources

ADAMTS13 as a thromboinflammatory brake at the vascular-immune interface: from viral immunothrombosis to the tumor microenvironment [PDF]

Frontiers in Immunology
ADAMTS13 is conventionally regarded as the protease whose severe deficiency causes thrombotic thrombocytopenic purpura (TTP). By cleaving shear-unfolded ultra-large and high-molecular-weight von Willebrand factor (VWF), ADAMTS13 limits a platelet- and ...
Peng Huang, Xiaoyu Sun, Jie Peng, Wei Tian   +3 more
doaj   +2 more sources

Pathogenicity of Anti-ADAMTS13 Autoantibodies in Acquired Thrombotic Thrombocytopenic Purpura

EBioMedicine, 2015
Background: Acquired thrombotic thrombocytopenic purpura (TTP) is an autoimmune disease in which anti-ADAMTS13 autoantibodies cause severe enzyme deficiency.
Mari R. Thomas, Rens de Groot, Marie A. Scully, James T.B. Crawley   +3 more
doaj   +3 more sources

Cancer-Related Microangiopathic Haemolytic Anemia Revealing Occult Metastatic Gastrointestinal Signet-Ring Cell Carcinoma: A Clinicopathological Case Report and Practical Differential Diagnostic Approach. [PDF]

Clin Case Rep
ABSTRACT This case highlights key laboratory red flags and a failure to respond to standard treatment, both of which should prompt urgent investigation for occult malignancy in patients presenting with cancer‐related microangiopathic hemolytic anemia (CR‐MAHA).
Jovanovic D, Sretenovic S, Jovanovic D, Ilic M, Djurdjevic P.   +4 more
europepmc   +2 more sources

Association between plasma ADAMTS13 levels/activity and the risk of ischemic stroke and outcomes of recanalization therapies: a systematic review and meta-analysis [PDF]

Frontiers in Neurology
BackgroundLarge vWF (von Willebrand Factor) multimers are associated with ischemic stroke, and itsthrombogenicity is controlled by ADAMTS13 (a Disintegrin and Metalloprotease with repetitions in the ThromboSpond in motif).
Yanyan Li, Rui Ding, Peng Gao, Ziqi Liu, Xiaorui Pei, Lifeng Piao   +5 more
doaj   +2 more sources

Characterization of ADAMTS13 and von Willebrand factor levels in septic and non-septic ICU patients.

PLoS ONE, 2021
Sepsis is a life-threatening disease characterized by excessive host response to infection that can lead to activation of the coagulation system. Von Willebrand Factor (VWF) and ADAMTS13 are important regulators of hemostasis and their dysregulation ...
Kanwal Singh, Andrew C Kwong, Hasam Madarati, Sharumathy Kunasekaran, Taylor Sparring, Alison E Fox-Robichaud, Patricia C Liaw, Colin A Kretz   +7 more
doaj   +1 more source

The Ratio of von Willebrand Factor Antigen to ADAMTS13 Activity: Usefulness as a Prognostic Biomarker in Acute-on-Chronic Liver Failure

Biology, 2023
Acute-on-chronic liver failure (ACLF) has a high risk of short-term mortality. A disintegrin-like and metalloproteinase with thrombospondin type-1 motifs 13 (ADAMTS13) is a metalloproteinase that specifically cleaves multimeric von Willebrand factor (VWF)
Hiroaki Takaya, Tadashi Namisaki, Masahide Enomoto, Takahiro Kubo, Yuki Tsuji, Yukihisa Fujinaga, Norihisa Nishimura, Kosuke Kaji, Hideto Kawaratani, Kei Moriya, Takemi Akahane, Masanori Matsumoto, Hitoshi Yoshiji   +12 more
doaj   +1 more source