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...

...ngs with timely access to plasma ADAMT...


...1. Diagnosis in settings with timely access to...


...with timely access to plasma ADAMTS13 testing an...


...able 2. Diagnosis in settings with ti...


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


...le 3. PLASMIC score or French score...


.... A suggested diagnostic and management s...


Figure 2. A suggested diagnostic and man...


Treatment

...atment...

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

...ients with iTTP experiencing a first acu...

...tients with iTTP experiencing their firs...

...s with iTTP experiencing a relapse, t...

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

...ith iTTP experiencing an acute event (first ev...

...patients with iTTP who are in remissio...


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

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

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

For patients with iTTP who are pregnan...

...th cTTP who are pregnant, the panel...

...th cTTP who are pregnant, the panel su...


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


...N I. Supportive Car...

...tatements pertain to initial emergency care an...

...tatement 1

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

Statement...

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

...atement 3...

...of suspicion for TTP is high, clini...

...tement 4

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

...tatement...

...t systematically search and review...

...atement...

...patients often need a central venous acc...

...atement 7

...isk of catheter related complications such a...

...atement 8...

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

...tement 9

...on indirect evidence in other critically il...

...tement 10...

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

...ement 11...

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


...ION II. Relapse Preventio...

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

...ement 12...

...systematically review the evidence on trigge...

Statemen...

➤ We did not systematically search and...


...II. Other treatment agents for TTP...

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

...tatement 1...

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

...ement 15...

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

...atement 16

...not systematically search and revi...

...ement 17...

...id not systematically search and rev...

Statement 1...

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

...ement 19...

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

Statement...

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

...atement 21...

...We did not systematically search...

...ement 22...

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


...TTP and Women’s Health...

➤ The following statements pertain to women's...

...tement 23

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

Statemen...

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

...tatement 2...

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

Statement 2...

...omen with a history of either cTTP or iTTP are us...

...tement 27...

...senting in pregnancy generally mer...

Statemen...

...th a history of iTTP require close mo...

...tatement...

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

...tement 3...

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

...atement 31

➤ We did not systematically search...

...tement 32...

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


...ECTION V. Refusal of Blo...

...g statement pertains to care of TTP in patients...

...tement 3...

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