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...

...n settings with timely access to plasma ADA...


...able 1. Diagnosis in settings with timely...


...with timely access to plasma ADAMTS13 tes...


...able 2. Diagnosis in settings with t...


...no reasonable access to plasma ADAMTS13 testin...


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


...re 1. A suggested diagnostic and management strat...


...gested diagnostic and management s...


Treatment

...eatment

...ediated TTP (iTTP)

...with iTTP experiencing a first acute...

For patients with iTTP experiencing their f...

...ith iTTP experiencing a relapse, the panel reco...

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

...h iTTP experiencing an acute event (first e...

...nts with iTTP who are in remission...


...tary or Congenital TTP (cTTP)...

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

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

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

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

For patients with cTTP who are pregnant,...


...uideline panel discussed a number of additional c...


...ECTION I. Supportive Car...

...e following statements pertain to initial emer...

...tement 1...

...generally consider a diagnosis of TT...

...atement 2

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

Statement 3

...¤ If the index of suspicion for TTP i...

...tatement...

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

...atement...

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

Statement...

...P patients often need a central venous access secu...

Statemen...

...of catheter related complications such as ble...

Statement...

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

...atement 9

...indirect evidence in other critically ill p...

Statement 10

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

...ement 11...

...not systematically search and revi...


...N II. Relapse Prevention...

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

...atement 12...

...ystematically review the evidence...

...tatement 13...

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


...ECTION III. Other treatment agents for TT...

...¤ The following nine statements per...

...atement 14...

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

...tatement...

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

...tement 16

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

...tatement...

...not systematically search and revi...

...ement 18...

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

...ement 19...

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

...ement 20...

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

Statemen...

...t systematically search and review the eviden...

...atement 22...

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


...TION IV. TTP and Women’s Heal...

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

...ement 23...

...h a history of TTP who are planning pregnan...

...tatement 24

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

...tatement 2...

...atients treated with rituximab are usu...

...atement...

...nt women with a history of either cTTP or iTTP...

...ement 27...

...senting in pregnancy generally merits transf...

...ement 28

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

...tatement 2...

...with a history of TTP who are planning pregnanc...

...tement 30...

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

...atement...

...not systematically search and review the e...

...ement 32...

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


...Refusal of Blood Products...

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

...tement 3...

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