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

...gnosis

...ngs with timely access to plasma ADAM...


...Diagnosis in settings with timely a...


...gs with timely access to plasma ADAMTS13 testi...


...2. Diagnosis in settings with tim...


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


...e 3. PLASMIC score or French score predicts the...


...suggested diagnostic and management strateg...


...suggested diagnostic and management strategy for...


Treatment

Treatmen...

...une-mediated TTP (iTTP)...

...ith iTTP experiencing a first acute event, the...

...s with iTTP experiencing their first acute eve...

...tients with iTTP experiencing a relapse, the pan...

...th iTTP experiencing a relapse, the pan...

...or patients with iTTP experiencing...

...th iTTP who are in remission, but...


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

...patients with cTTP who are in remission, the pan...

...h cTTP who are in remission, the panel suggest...

...ts with iTTP who are pregnant and have decrease...

...ents with cTTP who are pregnant, the...

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


...Guideline panel discussed a number of additional...


...N I. Supportive Car...

...owing statements pertain to initial emerge...

...atement 1...

...generally consider a diagnosis of...

...tement 2...

...ystematically search and review th...

...tement 3...

...the index of suspicion for TTP is high, clin...

...tatement 4...

...he severity and instability of their illne...

...tatement 5

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

Statemen...

...ients often need a central venous access se...

...tatement...

...ž¤ The risk of catheter related complications such...

...tement 8...

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

Statemen...

...ased on indirect evidence in other c...

Statement 1...

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

...atement 11...

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


SECTION II. Relapse Preventio...

...is section, the panel provides state...

...tement 1...

...We did not systematically review the evidence...

...atement 13...

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


...Other treatment agents for TTP...

...ing nine statements pertain to the use...

...ement 14...

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

...atement...

...did not systematically search and review...

Statement 16

...systematically search and review the ev...

...ement 17...

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

...atement 18

...t systematically search and review the evidence o...

Statement 19

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

...ement 20...

...ystematically search and review th...

...atement...

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

...ement 22...

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


.... TTP and Women’s Health...

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

...ement 23

...with a history of TTP who are plann...

Statemen...

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

...tement 25...

...s treated with rituximab are usually ask...

...tement 26

➤ Pregnant women with a history of eith...

Statement 27

...senting in pregnancy generally merits transfer...

...tement 28

...a history of iTTP require close monitoring by...

...tatement 29...

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

...tatement 30

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

...tatement 31...

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

...tatement...

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


SECTION V. Refusal of Blood Produ...

...The following statement pertains to...

...tatement 3...

...systematically search and review the...