Reduced ADAMTS13 activity during TTP remission is associated with stroke in TTP survivors

ADAMTS13, TTP and Beyond

ADAMTS13 (A Disintegrin And Metalloprotease with ThromboSpondin Type 1 repeats, 13), a plasma metalloprotease, cleaves von Willebrand factor (VWF) [1,2]. This cleavage is crucial for reducing the size of VWF multimers and adhesiveness, thereby preventing excessive platelet aggregation and thrombus formation at sites of vascular injury [3,4]. Deficiency of plasma ADAMTS13 activity could result i...

Acquired TTP: ADAMTS13 meets the immune system.

The majority of the patients affected by acquired thrombotic thrombocytopenic purpura (TTP) develop autoantibodies directed towards ADAMTS13 that interfere with its von Willebrand Factor (VWF) processing activity. B cell responses have been shown to primarily target the spacer domain of ADAMTS13 thereby prohibiting the binding of ADAMTS13 to the VWF A2 domain. In this review we summarize recent...

TTP-HUS associated with sunitinib.

Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) is a rare condition that is severe and may be fatal. Adverse reactions to drugs increasingly are reported as probable causes of TTP-HUS. Many chemotherapeutic agents have also been implicated in causing TTP-HUS. We reported a woman with metastatic renal cell carcinoma who presented with TTP-HUS associated with sunitinib. Sh...

The role of ADAMTS13 in the new pathogenesis of TTP.

Thrombotic Thrombocytopenic Purpura (TTP) is a severe microvascular occlusive microangiopathy characterized by thrombocytopenia, Coombs-negative hemolytic anemia and ischemic symptoms localized mainly but not exclusively in central nervous system and resulting from diffuse platelet thrombi in microcirculation [1]. To understand the broad clinical spectrum of TTP it is important to read the orig...

Clinical Policy Bulletin: ADAMTS13 Assay for Thrombotic Thrombocytopenic Purpura (TTP)