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

Diagnosi...

...ngs with timely access to plasma ADAMTS13 activi...


...osis in settings with timely access to pl...


...n settings with timely access to pl...


...ble 2. Diagnosis in settings with timely access...


...f no reasonable access to plasma ADAMT...


...C score or French score predicts the likeli...


...e 1. A suggested diagnostic and manag...


...re 2. A suggested diagnostic and managem...


Treatment

...eatmen...

Immune-mediated TTP...

...patients with iTTP experiencing a fi...

...patients with iTTP experiencing their fi...

...r patients with iTTP experiencing a relapse, the...

...h iTTP experiencing a relapse, the panel...

...tients with iTTP experiencing an acute event (firs...

...ients with iTTP who are in remission,...


...ditary or Congenital TTP (cTTP)...

For patients with cTTP who are in remission, the p...

...th cTTP who are in remission, the panel sugges...

...ents with iTTP who are pregnant and have...

...ts with cTTP who are pregnant, the p...

...ients with cTTP who are pregnant, the panel sugges...


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


...N I. Supportive Ca...

...atements pertain to initial emergency...

...tement 1...

...inicians generally consider a diagnosis of TTP...

Statemen...

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

...tement 3

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

...atement 4...

...everity and instability of their illness, TTP p...

...tement 5...

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

Statement...

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

...atement 7

...isk of catheter related complications such as bl...

Statemen...

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

...atement 9

...Based on indirect evidence in other critical...

...ement 10...

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

Statemen...

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


...II. Relapse Prevention...

...section, the panel provides statements...

...tatement 1...

...did not systematically review the evidence o...

...tatement 13

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


...Other treatment agents for TTP...

...ng nine statements pertain to the use of TTP trea...

...ement 14

...ot systematically search and review t...

...tatement 15...

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

...atement 16...

➤ We did not systematically search and revie...

Statement 1...

...not systematically search and review...

...tement 18...

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

...ement 19...

...systematically search and review the...

...tement 2...

...systematically search and review the e...

...ement 21...

...d not systematically search and review the eviden...

Statement...

➤ We did not systematically search and review...


...TTP and Women’s Health...

...owing statements pertain to women's health is...

Statement 23

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

...tement 2...

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

...ement 25...

...atients treated with rituximab are us...

...tatement 26...

...nant women with a history of either cTTP...

...ement 27...

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

...tatement...

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

...atement 29

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

...tement 30...

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

...atement 3...

...systematically search and review the e...

...tatement 32

➤ We did not systematically search and revie...


...V. Refusal of Blood Prod...

➤ The following statement pertains t...

...tement 33...

...not systematically search and review...