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. 'Fast Facts: Thrombotic Thrombocytopenic Purpura' sets
out in a clear and accessible format the steps to suspecting diagnosing and treating this
potentially devastating disease. These steps are complemented by clear descriptions of the
disease mechanism and epidemiology. Differential diagnosis which is of the utmost importance
for this disease is explored in detail. Table of Contents: -Disease overview -Clinical
presentation -Differential diagnosis -Laboratory findings and diagnosis -Management
