Thrombotic thrombocytopenic purpura: pathophysiology, diagnosis, and management

S Sukumar, B Lämmle, SR Cataland - Journal of clinical medicine, 2021 - mdpi.com
Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy
characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and …

Thrombotic thrombocytopenic purpura

JA Kremer Hovinga, P Coppo, B Lämmle… - Nature reviews Disease …, 2017 - nature.com
Thrombotic thrombocytopenic purpura (TTP; also known as Moschcowitz disease) is
characterized by the concomitant occurrence of often severe thrombocytopenia …

ADAMTS13 and von Willebrand factor in thrombotic thrombocytopenic purpura

XL Zheng - Annual review of medicine, 2015 - annualreviews.org
Pathogenesis of thrombotic thrombocytopenic purpura (TTP) was a mystery for over half a
century until the discovery of ADAMTS13. ADAMTS13 is primarily synthesized in the liver …

The diagnosis and management of the haematologic manifestations of lupus

A Velo-García, SG Castro, DA Isenberg - Journal of autoimmunity, 2016 - Elsevier
Haematological manifestations in systemic lupus erythematosus (SLE) are frequently
observed. They are diverse and range from mild to severe. Therefore, different treatment …

An update on pathogenesis and diagnosis of thrombotic thrombocytopenic purpura

BS Joly, P Coppo, A Veyradier - Expert review of hematology, 2019 - Taylor & Francis
Introduction: Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening
thrombotic microangiopathy characterized by microangiopathic hemolytic anemia …

The expanding field of IgG4‐mediated neurological autoimmune disorders

MG Huijbers, LA Querol, EH Niks… - European journal of …, 2015 - Wiley Online Library
At least 13 different disease entities affecting the central nervous system, peripheral nervous
system and connective tissue of the skin or kidneys are associated with immunoglobulin G4 …

Structure–function and regulation of ADAMTS‐13 protease

XL Zheng - Journal of Thrombosis and Haemostasis, 2013 - Wiley Online Library
Summary ADAMTS‐13, a plasma reprolysin‐like metalloprotease, cleaves von W illebrand
factor (VWF). Severe deficiency of plasma ADAMTS‐13 activity results in thrombotic …

Pathogenicity of anti-ADAMTS13 autoantibodies in acquired thrombotic thrombocytopenic purpura

MR Thomas, R de Groot, MA Scully, JTB Crawley - EBioMedicine, 2015 - thelancet.com
Background Acquired thrombotic thrombocytopenic purpura (TTP) is an autoimmune
disease in which anti-ADAMTS13 autoantibodies cause severe enzyme deficiency …

[HTML][HTML] An open conformation of ADAMTS‐13 is a hallmark of acute acquired thrombotic thrombocytopenic purpura

E Roose, AS Schelpe, BS Joly, M Peetermans… - Journal of Thrombosis …, 2018 - Elsevier
Essentials• Conformational changes in ADAMTS‐13 are part of its mode‐of‐action.• The
murine anti‐ADAMTS‐13 antibody 1C4 discriminates between folded and open ADAMTS …

Gain-of-function ADAMTS13 variants that are resistant to autoantibodies against ADAMTS13 in patients with acquired thrombotic thrombocytopenic purpura

C Jian, J Xiao, L Gong, CG Skipwith… - Blood, The Journal …, 2012 - ashpublications.org
Thrombotic thrombocytopenic purpura (TTP) is primarily caused by immunoglobulin G (IgG)
autoantibodies against A Disintegrin And Metalloprotease with ThromboSpondin type 1 …