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

...iagnosis

...tings with timely access to plasma ADAMTS13 acti...


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


...h timely access to plasma ADAMTS13 te...


Table 2. Diagnosis in settings with...


...of no reasonable access to plasma A...


...score or French score predicts the...


...uggested diagnostic and management s...


...re 2. A suggested diagnostic and mana...


Treatment

Treatment

...e-mediated TTP (iTTP)...

...nts with iTTP experiencing a first acute event,...

...s with iTTP experiencing their first acute event,...

...ients with iTTP experiencing a relapse, the pane...

...atients with iTTP experiencing a relapse...

...tients with iTTP experiencing an acute ev...

...r patients with iTTP who are in remission, but st...


...or Congenital TTP (cTTP)...

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

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

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

...r patients with cTTP who are pregnant, the pan...

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


...he Guideline panel discussed a number...


...I. Supportive Care...

...statements pertain to initial emergency care...

...tatement...

...icians generally consider a diagnosis of TTP in in...

...tement 2...

...ot systematically search and review the eviden...

...tatement 3

...If the index of suspicion for TTP is high, clini...

...tement 4

... Due to the severity and instabilit...

...tement 5...

➤ We did not systematically search and revie...

...tatement 6

...TTP patients often need a central venous acces...

Statement...

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

Statemen...

...systematically search and review th...

...tement 9...

...ndirect evidence in other critically ill patient...

...tatement 10

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

...ement 11

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


...II. Relapse Prevention

...In this section, the panel provide...

...tement 12

...systematically review the evidence on trigg...

...tement 13

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


...I. Other treatment agents for TT...

...The following nine statements pertain to the u...

...ement 14...

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

...tement 15...

...systematically search and review the evidenc...

...atement 1...

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

Statement 17

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

Statement...

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

...ement 19...

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

...ement 20...

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

...tatement 21...

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

...tement 22...

...t systematically search and review...


...IV. TTP and Women’s Health

...g statements pertain to women's health issues in...

...ement 23...

... Women with a history of TTP who are plan...

...atement...

...institutions, women with a decreased ADAMTS13...

Statemen...

...ients treated with rituximab are usuall...

...tatement 26...

...nant women with a history of either cTTP...

...tement 2...

... TTP presenting in pregnancy generally merits...

...ement 28...

...ith a history of iTTP require close monitoring b...

Statement...

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

...tatement...

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

...ement 31...

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

...tement 32...

...ot systematically search and review the evi...


...ION V. Refusal of Blood Products

...lowing statement pertains to care of TTP in patien...

...atement 33...

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