Thrombotic thrombocytopenic purpura--the role of ADAMTS13 assay in clinical practice.

Report of two unrelated cases of Familial Thrombotic Thrombocytopeic Purpura

Thrombotic Thrombocytopenic Purpura (TTP) is a rare microangiopathic disorder characterised by the pentad of microangiopathic hemolytic anemia, thrombocytopenic purpura, neurologic abnormalities, fever, and renal disease. Decreased production and/or activity of ADAMTS13 is the cause of this disorder. ADAMTS13 is a metalloproteinase which is responsible of the cleavage of high weight multimers o...

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

A Case Report of Successful Management of Thrombotic Thrombocytopenic Purpura Syndrome (TTP) in a Pregnant Woman with Plasmapheresis

Background and Aim: Thrombotic thrombocytopenic purpura is a rare and life-threatening disease with a relatively high prevalence of 5% in pregnancy that causes thrombocytopenia. Due to the high prevalence and mortality of fetal and maternal mortality, which resolves rapidly with the onset of plasmapheresis, the diagnosis of this disease is clinically important. The aim of this study was to succ...

Thrombotic thrombocytopenic purpura in a child with systemic lupus erythematosus.

We report a child with thrombotic thrombocytopenic purpura (TTP) secondary to systemic lupus erythematosus. The diagnosis was confirmed by low ADAMTS13 activity (<5%) along with the presence of a low titer inhibitor. Her clinical course was complicated by systemic lupus erythematosus, immunosuppressant therapy, and septic shock. She responded to plasma exchange and ADAMTS13 activity levels reco...

ADAMTS13 activity and antigen during therapy and follow-up of patients with idiopathic thrombotic thrombocytopenic purpura: correlation with clinical outcome.

BACKGROUND The assay for ADAMTS13 activity helps clinicians to confirm the clinical diagnosis of idiopathic thrombotic thrombocytopenic purpura. The clinical value of testing for the antigen level of ADAMTS13 protein is, however, less clear. DESIGN AND METHODS In this study, both ADAMTS13 antigen and activity levels were measured in 835 sequential samples from 40 consecutive patients who were...