The prevalence, incidence and outcomes of haemolytic uraemic syndrome (HUS) and thrombotic thrombocytopaenic purpura (TTP) are not well established in adults or children from prospective studies. We sought to identify both outcomes and current management strategies using prospective, national surveillance of HUS and TTP, from 2003 to 2005 inclusive. We also investigated the links between these ...

AKI in pregnancy remains a cause of significant fetomaternal mortality and morbidity, particularly in developing countries. Hypertensive complications of pregnancy (preeclampsia/eclampsia or hemolysis, elevated liver enzymes, and low platelets count syndrome) are the leading cause of AKI in pregnancy worldwide. Thrombotic microangiopathy is another peculiar and devastating cause of AKI in pregn...

Anti-cardiolipin antibodies have been linked to recurrent arterial and venous thrombosis in multiple organs. We present a biopsy-documented report of thrombotic renal disease apparently attributable to circulating anti-cardiolipin antibodies. One patient had primary anti-cardiolipin syndrome, one had mild SLE, and the third had a mild lupus-like syndrome. All three patients had a clinical cours...

Welcome to this special issue of Seminars in Thrombosis & Hemostasis, which summarizes up-to-date knowledge on pathogenesis, diagnosis, and treatment of thrombotic microangiopathies (TMAs) such as (enterohemorrhagic) E. coli (EHEC)–associated hemolytic uremic syndrome (HUS) (eHUS), atypical HUS (aHUS), (aHUS) and thrombotic thrombocytopenic purpura (TTP). This issue also reviews C3 glomerulopat...

Thromb Haemost 2005;3:1432-6. 4. Dashe JS, Ramin SM, Cunningham FG. The long-term consequences of thrombotic microangiopathy (thrombotic thrombocytopenic purpura and hemolytic uremic syndrome) in pregnancy. Obstet Gynecol 1998;91:662-8. 5. Miner PE, Nutt RL, Thomas ME. Thrombotic thrombocytopenic purpura occurring in pregnancy. Am J Obstet Gynecol 1955;70:611-7. 6. Weiner CP. Thrombotic microan...

Von Willebrand Factor (VWF) is a multimeric glycoprotein that plays a major role in platelet adhesion and aggregation. Large VWF multimers are highly procoagulant and are cleaved by its metalloprotease ADAMTS13 (A Disintegrin And Metalloprotease with ThromboSpondin motif repeats 13) in smaller, less procoagulant forms. In case of a deficiency of ADAMTS13, large VWF multimers are not cleaved, re...

Thrombocytopenia is one of the most common hematologic disorders, characterized by an abnormally low number of platelets from multiple causes. The normal count of thrombocytes (platelets) is between 150,000 and 450,000 per microliter. The clinical expression of thrombocytopenia has broad variation from asymptomatic to life-threatening bleeding. Various syndromes and diseases are associated with...

PURPOSE Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder and early treatment is vital. Here, we review the recent advances in the understanding of the pathophysiology of TTP and its treatment. CURRENT KNOWLEDGE AND KEY POINTS Recent advances have shown that TTP is caused by deficiency of the (ADAMTS-13) metalloprotease that cleaves von Willebrand factor multimers. Acqu...

Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening complication of gemcitabine treatment. Since the approval of this nucleoside analog for the treatment of pancreatic cancer by the FDA in 1996, reported incidence varies from 0.015 to 1.4%. The classic 'pentad' describing the disease process (fever, hemolytic anemia, thrombocytopenia, neurological complications and renal im...