[HTML][HTML] Thrombotic thrombocytopenic purpura: pathogenesis, diagnosis and potential novel therapeutics

M Saha, JK McDaniel, XL Zheng - Journal of Thrombosis and Haemostasis, 2017 - Elsevier
Thrombotic thrombocytopenic purpura (TTP), a potentially fatal clinical syndrome, is
primarily caused by autoantibodies against the von Willebrand factor (VWF)‐cleaving …

Thrombotic microangiopathies: a general approach to diagnosis and management

DM Arnold, CJ Patriquin, I Nazy - Cmaj, 2017 - Can Med Assoc
ADAMTS13, and the inherited form is due to a genetic deficiency of ADAMTS13. In 1996,
Furlan and colleagues28 and Tsai29 independently found that patients with TTP had …

Laboratory testing for ADAMTS13: utility for TTP diagnosis/exclusion and beyond

EJ Favaloro, L Pasalic, B Henry… - American journal of …, 2021 - Wiley Online Library
The metalloproteinase ADAMTS13 (a disintegrin with a thrombospondin type 1 motif,
member 13), also known as VWF (von Willebrand factor) protease, may be assessed in a …

Atypical hemolytic uremic syndrome in the setting of complement-amplifying conditions: case reports and a review of the evidence for treatment with eculizumab

A Asif, A Nayer, CS Haas - Journal of nephrology, 2017 - Springer
Atypical hemolytic uremic syndrome (aHUS) is a rare, genetic, progressive, life-threatening
form of thrombotic microangiopathy (TMA) predominantly caused by dysregulation of the …

The role of ADAMTS13 testing in the diagnosis and management of thrombotic microangiopathies and thrombosis

C Masias, SR Cataland - Blood, The Journal of the American …, 2018 - ashpublications.org
ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motif, 13) is a
metalloprotease responsible for cleavage of ultra-large von Willebrand factor (VWF) …

[HTML][HTML] A multicenter laboratory assessment of a new automated chemiluminescent assay for ADAMTS13 activity

EJ Favaloro, S Mohammed, K Chapman… - Journal of Thrombosis …, 2021 - Elsevier
Background Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially fatal
disorder caused by ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin …

ADAMTS13: origins, applications, and prospects

WE Plautz, JS Raval, MR Dyer, MA Rollins‐Raval… - …, 2018 - Wiley Online Library
ADAMTS13 is an enzyme that acts by cleaving prothrombotic von Willebrand factor (VWF)
multimers from the vasculature in a highly regulated manner. In pathologic states such as …

[HTML][HTML] The standard of care for immune thrombotic thrombocytopenic purpura today

XL Zheng - Journal of Thrombosis and Haemostasis, 2021 - Elsevier
Targeted therapy of immune thrombotic thrombocytopenic purpura (iTTP) requires acurate
and prompt diagnosis and differentiation from complement‐mediated hemolytic uremic …

Complement C5-inhibiting therapy for the thrombotic microangiopathies: accumulating evidence, but not a panacea

V Brocklebank, D Kavanagh - Clinical kidney journal, 2017 - academic.oup.com
Thrombotic microangiopathy (TMA), characterized by organ injury occurring consequent to
severe endothelial damage, can manifest in a diverse range of diseases. In complement …

Thrombotic Microangiopathy Syndromes—Common Ground and Distinct Frontiers

RM Hanna, K Henriksen, K Kalantar-Zadeh… - Advances in chronic …, 2022 - Elsevier
Thrombotic microangiopathies (TMAs) have in common a terminal phenotype of
microangiopathic hemolytic anemia with end-organ dysfunction. Thrombotic …