Diagnosis of Thrombotic Thrombocytopenic Purpura

Publication Date: July 1, 2020

Key Points

Key Points

  • Remarkable advances have been made in recent years towards our understanding of the pathophysiology of thrombotic thrombocytopenic purpura (TTP), which has led to advancements in novel therapies.
  • Despite an increase in our understandings of pathogenesis of TTP, the approaches for initial diagnosis and management of TTP vary significantly.
  • The guidelines are intended to support patients, clinicians, and other healthcare professionals in their decisions about the initial diagnosis and management of acute TTP.

Diagnosis

...agnosis

...ith timely access to plasma ADAMTS13 activit...


...1. Diagnosis in settings with tim...


...settings with timely access to plasma ADAMTS13 t...


...agnosis in settings with timely access to plasma...


...n settings of no reasonable access to plasma AD...


...PLASMIC score or French score pred...


...suggested diagnostic and management...


...uggested diagnostic and management strat...


Treatment

...reatment

...mediated TTP (iTTP)...

...atients with iTTP experiencing a first ac...

...tients with iTTP experiencing their first acut...

...patients with iTTP experiencing a relapse,...

...ts with iTTP experiencing a relapse, the...

...atients with iTTP experiencing an acute event (fi...

...h iTTP who are in remission, but still have...


...ereditary or Congenital TTP (cTTP...

...ients with cTTP who are in remissi...

...s with cTTP who are in remission, the pa...

...patients with iTTP who are pregnant...

...with cTTP who are pregnant, the panel recom...

...with cTTP who are pregnant, the panel suggest...


...deline panel discussed a number of additional...


...ECTION I. Supportive Care

...lowing statements pertain to initial emergenc...

...atement 1

...nicians generally consider a diagnosis of...

Statement...

...not systematically search and review the evidence...

Statemen...

...ndex of suspicion for TTP is high, clinicians sho...

Statemen...

...to the severity and instability of t...

Statement 5

...ystematically search and review the evide...

...tement 6...

...¤ TTP patients often need a central v...

...atement...

...of catheter related complications s...

Statemen...

...e did not systematically search and...

...tatement 9

...direct evidence in other critically il...

...tatement...

...ž¤ We did not systematically searc...

...atement...

...not systematically search and review the evide...


...CTION II. Relapse Preven...

...ection, the panel provides statements p...

...ement 12...

...d not systematically review the evidence on trigge...

...atement 13...

...id not systematically search and review...


...N III. Other treatment agents for...

➤ The following nine statements pertain...

...tement 14...

...¤ We did not systematically search an...

...atement...

...We did not systematically search and review the...

...ement 16...

...id not systematically search and review the eviden...

...tement 1...

...t systematically search and review...

...atement 18

...id not systematically search and review t...

...tement 19

...systematically search and review the e...

...tatement...

...ot systematically search and review the evidence...

...atement...

...We did not systematically search an...

...ement 22

...did not systematically search and rev...


.... TTP and Women’s Healt...

...following statements pertain to wom...

Statement 23

...with a history of TTP who are planning pregnancy...

...tement 24...

...itutions, women with a decreased ADAMTS13 ac...

...tement 25...

...ts treated with rituximab are usually asked to w...

...tement 26

...Pregnant women with a history of either cTT...

...tatement 27...

...P presenting in pregnancy generally merits transfe...

...ement 28...

...h a history of iTTP require close...

...ement 29...

...omen with a history of TTP who are plannin...

Statemen...

...We did not systematically search and review the...

...atement 31

...did not systematically search and r...

Statement 32

...systematically search and review t...


...TION V. Refusal of Blood Prod...

...following statement pertains to care of TTP in pa...

...tement 33...

...not systematically search and revie...