Thrombotic thrombocytopenic purpura: is there more than ADAMTS-13?

Recombinant ADAMTS 13 in thrombotic thrombocytopenic purpura

Thrombotic microangiopathies, thrombotic thrombocytopenic purpura, and ADAMTS-13.

Thrombotic microangiopathy (TMA) is a term used to describe a group of disorders characterized by hemolytic anemia (with prominent red blood cell fragmentation), thrombocytopenia, and thrombosis in the microvasculature. It may be used when describing patients with thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome, atypical hemolytic uremic syndrome, as well as a myriad of oth...

The von Willebrand factor-cleaving protease (ADAMTS-13) and the diagnosis of thrombotic thrombocytopenic purpura (TTP).

Thrombotic thrombocytopenic purpura (TTP) is a life threatening disorder characterized by microangiopathic hemolytic anemia and thrombocytopenia as a result of microvascular platelet clumping often accompanied by ischemic organ dysfunctions such as neurological abnormalities or renal insufficiency, and fever. Until the sixties of the 20th century TTP remained an almost universally fatal disorde...

Congenital Thrombotic Thrombocytopenic Purpura: Atypical Presentation and New ADAMTS 13 Mutation in a Tunisian Child

BACKGROUND Congenital deficiency of ADAMTS13 is characterized by systemic platelet clumping, hemolytic anemia and multiorgan failure. Although, more than 100 mutations have been reported, atypical clinical presentation may be involved in diagnostic difficulties. CASE REPORT A 2 year old Tunisian child presented with chronic thrombopenic purpura which failed to respond to corticosteroids. Hemo...

Thrombotic Thrombocytopenic Purpura

Thrombotic microangiopathy comprises a spectrum of clinical and lab findings including microangiopathic hemolytic anemia, thrombocytopenia, and thrombosis of capillary and arterioles. Platelet and hyaline thrombi with complete or partial occlusion of these vessels are integral histopathological findings. These findings are seen irrespective of cause and organ involved. Pathogenesis and prognosi...