Abstract
While clinical recognition of thrombotic thrombocytopenia purpura (TTP) has been evident
for almost 90 years, the pathological basis of this disorder has only, relatively,
recently been elucidated. Consequently, options for treating TTP had evolved rather
slowly for many years. Despite this, current treatment practices of intensive plasma
exchange often with immune modulation have seen survival rates increase dramatically.
Nevertheless, the current understanding of TTP may witness the cusp of a new era for
this disorder, with new and emerging treatments nearing clinical practice that specifically
target the root cause of TTP. Some of these targeted approaches may even see the beginning
of plasma-free treatments for TTP, with potentially faster recoveries and fewer long-term
adverse effects.
Keywords
thrombotic thrombocytopenia purpura - TTP - therapy - von Willebrand cleaving protease
- ADAMTS-13
