TY  - BOOK
AU  - Scully,Marie A.
AU  - Cataland,Spero R.
TI  - Thrombotic thrombocytopenic purpura
T2  - Fast facts
SN  - 9781912776801 (PDF ebook) :
PY  - 2020///
CY  - Abingdon, Oxford
PB  - Karger
KW  - Thrombotic thrombocytopenic purpura
KW  - Purpura, Thrombotic Thrombocytopenic
N1  - Also issued in print: 2020; Includes bibliographical references and index; Specialized
N2  - Thrombotic thrombocytopenic purpura (TTP) is a rare disorder of the blood coagulation system. In most cases, a lack of the ADAMTS13 enzyme leads to an accumulation of ultra-large von Willebrand factor molecules in the plasma which, in turn, initiate the formation of microscopic thromboses in small blood vessels. TTP is a medical emergency. Timely diagnosis and urgent and effective management are vital - mortality in those untreated is in the region of 90%. The understanding of TTP pathogenesis has increased markedly in recent decades. It is now known that TTP is acquired (immunemediated) or congenital, and that the most common type - the acquired form - predominantly affects women in their 40s. It is also clear that the prompt delivery of plasma exchange saves lives
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