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
...agnosis...
...ngs with timely access to plasma ADAMTS13 ac...
Table 1. Diagnosis in settings with timely acce...
...h timely access to plasma ADAMTS13 testing and fo...
...e 2. Diagnosis in settings with timely access to...
...no reasonable access to plasma ADA...
...able 3. PLASMIC score or French score pred...
...re 1. A suggested diagnostic and managemen...
...ested diagnostic and management strate...
Treatment
...eatment...
...mediated TTP (iTTP)
...h iTTP experiencing a first acute event, the...
...with iTTP experiencing their first...
...or patients with iTTP experiencing a relap...
...ts with iTTP experiencing a relapse, the panel s...
...atients with iTTP experiencing an...
...patients with iTTP who are in remiss...
...ry or Congenital TTP (cTTP)...
...ients with cTTP who are in remission, the panel...
...atients with cTTP who are in remission, the panel...
...with iTTP who are pregnant and have...
...tients with cTTP who are pregnant, the...
...h cTTP who are pregnant, the panel sugg...
...MENTThe Guideline panel discussed a numbe...
...ECTION I. Supportive Car...
...ing statements pertain to initial emergency ca...
...tatement 1
...s generally consider a diagnosis of TTP in individ...
...atement 2...
...id not systematically search and revi...
Statement...
...dex of suspicion for TTP is high, cli...
...atement 4
...severity and instability of their illn...
Statement 5
...ystematically search and review the evidence...
...atement...
...atients often need a central venous a...
Statemen...
...of catheter related complications...
...tement 8
...e did not systematically search and r...
...tement 9...
...ž¤ Based on indirect evidence in other critically...
...atement 10...
...systematically search and review the...
...tatement...
➤ We did not systematically search and...
...ECTION II. Relapse Prev...
...s section, the panel provides statements pertaini...
Statement...
...systematically review the evidence on triggers f...
...tement 13...
➤ We did not systematically search and...
.... Other treatment agents for TTP...
...g nine statements pertain to the use of TTP t...
Statement...
...ystematically search and review the evid...
...atement...
...ystematically search and review the evidenc...
...ement 16...
...ot systematically search and review...
...tatement 1...
...id not systematically search and revi...
...tement 18
...d not systematically search and review the evide...
...ement 19...
...systematically search and review the evidence on a...
...tatement...
...ystematically search and review the evidence...
...tement 21...
...ž¤ We did not systematically search and r...
...tement 22
...did not systematically search and...
...IV. TTP and Women’s Health...
...he following statements pertain to women's...
...tement 2...
...a history of TTP who are planning pre...
...ement 24...
...stitutions, women with a decreased ADAMTS13 activi...
...tatement 2...
➤ Patients treated with rituxima...
Statement 2...
...omen with a history of either cTTP or iTTP are usu...
...tement 27...
...¤ TTP presenting in pregnancy gener...
...atement 28...
...Women with a history of iTTP require close mo...
...tatement 29...
...Women with a history of TTP who are planning pre...
...ement 30...
...did not systematically search and review the evi...
...atement 3...
...e did not systematically search an...
...ement 32...
...d not systematically search and re...
...ON V. Refusal of Blood Produ...
...llowing statement pertains to care...
Statement...
...did not systematically search and revi...