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...

...h timely access to plasma ADAMTS13 activity tes...


...able 1. Diagnosis in settings with tim...


In settings with timely access to plasma ADAMTS13...


...iagnosis in settings with timely acc...


...s of no reasonable access to plasma ADAMTS13 t...


...PLASMIC score or French score predict...


...A suggested diagnostic and management...


...e 2. A suggested diagnostic and management st...


Treatment

...reatment...

...mmune-mediated TTP (iTTP)

...atients with iTTP experiencing a first acute e...

...r patients with iTTP experiencing the...

...nts with iTTP experiencing a relapse, the pan...

...ients with iTTP experiencing a rel...

...ents with iTTP experiencing an acute event (fi...

...th iTTP who are in remission, but...


...y or Congenital TTP (cTTP)

...or patients with cTTP who are in remi...

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

...tients with iTTP who are pregnant and have decrea...

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

...s with cTTP who are pregnant, the panel su...


...deline panel discussed a number of additional...


...ION I. Supportive Care...

...g statements pertain to initial emergency...

...tement 1...

...Clinicians generally consider a diagnosis o...

...atement 2...

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

...atement 3...

...ex of suspicion for TTP is high, cl...

...tatement...

...ue to the severity and instability...

Statement...

➤ We did not systematically search...

...tement 6

...ž¤ TTP patients often need a central venous ac...

Statement...

...of catheter related complications such as bleedi...

...tement 8...

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

...atement 9...

...ed on indirect evidence in other cri...

...tement 10...

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

...tement 1...

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


...II. Relapse Prevention...

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

Statement 12

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

...tement 13

...did not systematically search and...


...I. Other treatment agents for TTP...

...lowing nine statements pertain to...

...tement 14...

...systematically search and review the...

...atement 15

...not systematically search and rev...

...tatement 16...

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

...ement 17...

...systematically search and review the evi...

Statement 1...

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

...ement 19...

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

...atement 20

...ystematically search and review the evidence on ec...

...tement 21...

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

Statement 2...

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


...IV. TTP and Women’s Health...

...following statements pertain to women's healt...

...ement 23...

...Women with a history of TTP who are planning preg...

Statement 2...

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

...atement 25...

...ž¤ Patients treated with rituximab are us...

...ement 26...

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

...ement 27...

...nting in pregnancy generally merits transfer t...

...atement...

...Women with a history of iTTP require close...

Statemen...

➤ Women with a history of TTP who are planning...

...ement 30...

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

...tement 31...

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

...atement 32...

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


...Refusal of Blood Products...

...¤ The following statement pertains to care of TTP...

...tatement...

...ystematically search and review the evidence on a...