ABSTRACT
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease that is caused by severe ADAMTS-13 deficiency. Immune-mediated TTP develops due to autoantibodies against ADAMTS-13, whereas congenital TTP is caused by mutations in the ADAMTS13 gene. Diagnostic possibilities and treatment options in TTP have emerged in recent years, which prompted the International Society on Thrombosis and Haemostasis (ISTH) to publish clinical practice guidelines for the diagnosis and treatment of TTP in 2020. In this article, the European Renal Best Practice Working Group endorsed the ISTH guidelines and emphasizes a number of considerations, including the importance of rapid ADAMTS-13 activity testing, the use of rituximab and anti-von Willebrand factor therapies such as caplacizumab, that enhance the clinical applicability of the guidelines in Europe.
Subject(s)
Purpura, Thrombotic Thrombocytopenic , Thrombosis , ADAMTS13 Protein , Hemostasis , Humans , Purpura, Thrombotic Thrombocytopenic/diagnosis , Purpura, Thrombotic Thrombocytopenic/etiology , Purpura, Thrombotic Thrombocytopenic/therapy , Thrombosis/diagnosis , Thrombosis/etiology , Thrombosis/therapy , von Willebrand FactorABSTRACT
Highlights In normoalbuminuric diabetic patients at low cardiovascular risk, the risk of transition from normo- to microalbuminuria is lower in women, despite the nonprotective effects of the angiotensin receptor blocker olmesartan. Additional methods of assessment of albuminuria in clinical studies (eg, measurements of albumin and creatinine excretion rate) should be implemented or the actually accepted higher urine albumin creatinine ratio (UACR) cutoff values for microalbuminuria in women reconsidered.
