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...

...tings with timely access to plasma ADAMTS1...


...is in settings with timely access to plasma AD...


...timely access to plasma ADAMTS13 test...


...is in settings with timely access to pla...


...ngs of no reasonable access to plasma...


...ASMIC score or French score predicts...


...suggested diagnostic and management strategy fo...


Figure 2. A suggested diagnostic and man...


Treatment

Treatm...

...mediated TTP (iTTP)...

...ts with iTTP experiencing a first acu...

...patients with iTTP experiencing the...

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

...or patients with iTTP experiencing...

...patients with iTTP experiencing an acut...

...ents with iTTP who are in remission, but st...


...editary or Congenital TT...

...nts with cTTP who are in remission, the...

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

...with iTTP who are pregnant and have d...

...patients with cTTP who are pregnant,...

...h cTTP who are pregnant, the panel sugges...


...he Guideline panel discussed a num...


...ION I. Supportive Care

...following statements pertain to i...

...atement 1...

...s generally consider a diagnosis of TTP in indi...

...tement 2...

... We did not systematically searc...

...atement 3

...ex of suspicion for TTP is high, clinicia...

...atement...

...ue to the severity and instability of their i...

...tement 5...

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

Statement 6

...atients often need a central venous access secur...

...atement 7...

...of catheter related complications such as...

...tement 8...

... We did not systematically search and...

...atement 9...

...d on indirect evidence in other critic...

Statement 10

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

...tement 11...

...not systematically search and review the evid...


...N II. Relapse Preventi...

...his section, the panel provides statements...

...tement 12...

...did not systematically review the evidenc...

...tement 13...

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


...ON III. Other treatment agents for...

...lowing nine statements pertain to the use o...

...atement 14...

...e did not systematically search and review the evi...

Statemen...

...t systematically search and review the...

...atement 16

...not systematically search and review the e...

Statement 1...

➤ We did not systematically search and review...

Statement...

...systematically search and review the...

...tement 1...

...d not systematically search and review the...

...tement 20...

...d not systematically search and review the...

...tatement 21...

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

...tement 22

...systematically search and review the evidence on...


...IV. TTP and Women’s He...

...wing statements pertain to women's health...

...ement 23...

...ith a history of TTP who are planning pregn...

...ement 24

...In some institutions, women with a decreased...

...tatement 25

...eated with rituximab are usually as...

...atement 26...

...men with a history of either cTTP or iTTP are u...

...tatement...

...TP presenting in pregnancy generally merits...

...ement 28...

...history of iTTP require close mon...

...tatement 29...

...en with a history of TTP who are planning pregn...

Statement...

...ystematically search and review the...

...tement 31...

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

...atement 32

...systematically search and review t...


...Refusal of Blood Products...

...llowing statement pertains to care of T...

...ement 33...

...not systematically search and review the ev...