Diagnosis of Thrombotic Thrombocytopenic Purpura
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...
...ttings with timely access to plasma ADAMTS13 act...
...is in settings with timely access to...
...h timely access to plasma ADAMTS13 testing...
Table 2. Diagnosis in settings with...
...ngs of no reasonable access to plasma ADAM...
...ble 3. PLASMIC score or French score predicts the...
...suggested diagnostic and management strat...
...2. A suggested diagnostic and manageme...
Treatment
Treatment
Immune-mediated TTP (iT...
...with iTTP experiencing a first acut...
...atients with iTTP experiencing their first acu...
...with iTTP experiencing a relapse,...
...atients with iTTP experiencing a relapse,...
...tients with iTTP experiencing an acute...
For patients with iTTP who are in remi...
...tary or Congenital TTP (cTT...
...atients with cTTP who are in remission, the...
...tients with cTTP who are in remiss...
For patients with iTTP who are pregnant and h...
...nts with cTTP who are pregnant, the panel recomm...
...or patients with cTTP who are pregnant,...
...ideline panel discussed a number of additiona...
...ON I. Supportive C...
...atements pertain to initial emergency ca...
...tement 1...
...¤ Clinicians generally consider a diagnosis of T...
Statement...
...did not systematically search and...
...tement 3...
...x of suspicion for TTP is high, clini...
Statement...
...everity and instability of their illnes...
...tatement...
...did not systematically search and r...
Statement...
...patients often need a central venous access s...
Statemen...
...The risk of catheter related complic...
...atement 8
...ž¤ We did not systematically search a...
...atement...
...irect evidence in other critically ill...
...tatement 10
...did not systematically search and revi...
...atement...
...id not systematically search and review the...
...I. Relapse Prevention...
...is section, the panel provides statements pertain...
...tatement 1...
...did not systematically review the evi...
...atement 13
...We did not systematically search and review the...
...II. Other treatment agents for TTP...
...e following nine statements pertain to...
...tement 1...
...systematically search and review the ev...
...ement 15...
...ystematically search and review the ev...
Statement 1...
...id not systematically search and review...
...ement 17...
...We did not systematically search an...
...tement 18...
...did not systematically search and revi...
...tement 19...
...not systematically search and review the eviden...
...tement 2...
➤ We did not systematically search and revi...
...atement 21...
...ystematically search and review the evidence on i...
...tement 22...
...ystematically search and review the evidence on c...
...CTION IV. TTP and Women’s...
...following statements pertain to women'...
...tatement 2...
...h a history of TTP who are planning pregnan...
...ement 24...
...ome institutions, women with a decreased...
...tement 25...
...reated with rituximab are usually asked to...
...atement...
...women with a history of either cT...
...tement 27...
...TP presenting in pregnancy generally merits trans...
...tatement 28...
...history of iTTP require close monitoring by a...
...tatement 2...
...Women with a history of TTP who are planning...
...tatement 3...
...¤ We did not systematically search and revi...
...ement 31
...ystematically search and review the eviden...
Statemen...
...not systematically search and review...
...TION V. Refusal of Blood Produc...
...wing statement pertains to care of TTP in patien...
...tement 33...
...t systematically search and review th...