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...

...with timely access to plasma ADAMTS13 activity t...


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


...ttings with timely access to plasma...


...le 2. Diagnosis in settings with timely access to...


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


...LASMIC score or French score predicts t...


...A suggested diagnostic and managemen...


...A suggested diagnostic and management strategy fo...


Treatment

...Treatmen...

...Immune-me...

...patients with iTTP experiencing a first acute ev...

...patients with iTTP experiencing their first acu...

...with iTTP experiencing a relapse, the pa...

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

...atients with iTTP experiencing an...

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


...Hereditary o...

...s with cTTP who are in remission, the panel sugge...

For patients with cTTP who are in remis...

...ts with iTTP who are pregnant and have dec...

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

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


...NTThe Guideline panel discussed a n...


...SECTION I. S...

...following statements pertain to initial...

...Stateme...

...ns generally consider a diagnosis of TTP in i...

...Stateme...

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

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

...Stat...

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

➤ We did not systematically search and review...

...Statement 6...

... TTP patients often need a centra...

...Stat...

...The risk of catheter related complications...

...Statement 8...

...d not systematically search and re...

...Statement 9...

...sed on indirect evidence in other critically il...

...Stateme...

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

...Statement 11...

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


...SECTION II. R...

...ction, the panel provides statements pertaining to...

...Statement...

➤ We did not systematically revie...

...Statement 1...

...did not systematically search and review t...


...SECTION III. Othe...

...following nine statements pertain to the u...

...Statement 1...

...did not systematically search and...

...State...

...not systematically search and revi...

...Statement 16...

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

...Statement 17...

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

...Statemen...

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

...Statement 19...

...id not systematically search and r...

...Statement...

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

...Statement 21

...ystematically search and review the evi...

...Statem...

...ystematically search and review the evi...


...SECTION...

...following statements pertain to wo...

...Statement 23

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

...Statement 24...

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

...State...

...treated with rituximab are usually aske...

...Statement...

...omen with a history of either cTTP or iTTP ar...

Statemen...

...P presenting in pregnancy generally meri...

...Statement...

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

...Statement 29...

...n with a history of TTP who are plann...

St...

➤ We did not systematically search...

...Statement 31...

...did not systematically search and...

...Stat...

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


...SECTION V. Refusal...

...ollowing statement pertains to care of T...

...Statement 3...

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