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

...timely access to plasma ADAMTS13 activity testi...


...le 1. Diagnosis in settings with timely...


...gs with timely access to plasma ADAMTS13 testing a...


...sis in settings with timely access...


...settings of no reasonable access to plas...


Table 3. PLASMIC score or French score p...


...A suggested diagnostic and management strateg...


...re 2. A suggested diagnostic and manag...


Treatment

...eatment

...mediated TTP (iTTP...

...ts with iTTP experiencing a first ac...

...with iTTP experiencing their first...

...with iTTP experiencing a relapse, th...

...atients with iTTP experiencing a relapse, the pa...

For patients with iTTP experiencing an acute even...

...atients with iTTP who are in remissi...


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

...tients with cTTP who are in remission, t...

...ients with cTTP who are in remission, th...

For patients with iTTP who are pregna...

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

...nts with cTTP who are pregnant, the panel suggests...


...line panel discussed a number of ad...


SECTION I. Supportive...

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

...atement...

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

...atement 2

...ystematically search and review the evidence...

...tatement...

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

...atement 4...

...the severity and instability of their ill...

...tement 5...

...ž¤ We did not systematically search a...

Statemen...

...tients often need a central venous a...

...atement...

➤ The risk of catheter related complications s...

Statement...

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

...atement 9...

...n indirect evidence in other critic...

...ement 10...

...systematically search and review th...

...tatement 11...

...ystematically search and review the evi...


...ECTION II. Relapse Preve...

...is section, the panel provides statements pe...

Statement 12

...ot systematically review the evidence on tr...

...atement 1...

...not systematically search and revie...


...ECTION III. Other treatment agents fo...

...ž¤ The following nine statements pe...

...ement 14...

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

...tement 15

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

...tatement 1...

...not systematically search and review th...

Statement...

...did not systematically search and...

Statement...

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

...ement 19...

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

...tatement...

...e did not systematically search an...

...ement 21

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

...atement 2...

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


...IV. TTP and Women’s He...

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

...tement 23

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

...tatement 24...

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

...ement 25...

...treated with rituximab are usually asked...

...atement 26...

...Pregnant women with a history of either cTTP or...

...ement 27...

...esenting in pregnancy generally merits transf...

...tement 28

...omen with a history of iTTP require close monitori...

...tement 29...

...th a history of TTP who are planni...

...tatement 30

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

...atement...

...not systematically search and revie...

...ement 32...

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


...Refusal of Blood Products...

...ž¤ The following statement pertains to care o...

...ement 33...

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