An Acute Febrile Pleiochromic Anemia With Hyaline Thrombosis of the Terminal Arterioles and Capillaries

Principles of Separation

“…ADAMTS13 is the vWf-cleaving metalloprotease that is deficient in activity either by a congenital genetic mutation or an acquired autoimmune condition in TTP (25,28,29). The low activity of this protease leads to large vWF multimers, which allow platelet aggregation in response to intravascular shear stress and form the critical nidus of microthrombi formation in the arterioles (31). A relatively large (102 patients) randomized controlled clinical trial has shown the superiority of TPE over plasma infusion alone to treat TTP (30).…”

Thrombotic Thrombocytopenic Purpura: A Thrombotic Disorder Caused by ADAMTS13 Deficiency

“…First reported in 1924 by Moschcowitz [1], thrombotic thrombocytopenic purpura (TTP) usually refers to the disorder of thrombocytopenia, hemolysis with schistocytes on blood smears, and neurologic abnormalities such as headache, confusion, focal deficits, seizures or coma. These manifestations are due to widespread microvascular thrombosis involving the capillaries and arterioles of the brain and other organs.…”

Thrombotic Microangiopathy-Kidneys and Beyond: Historic Lessons

“…The first description of the condition, belonging to the continuum of thrombotic microangiopathies, was published 1924, when Moschcowitz reported a case of acute febrile anemia with petechiae, renal involvement and hyalin thrombosis of the terminal arterioles and capillaries in adolescent girl [1,2]. The next case, reported by Baehr and co-workers in 1936, presented with reticulocytosis and thrombocytopenia in addition to severe anemia [3].…”