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

...Diagnosis...

...n settings with timely access to p...


...gnosis in settings with timely access to pla...


...n settings with timely access to plasma ADAMTS1...


...is in settings with timely access to pla...


...ings of no reasonable access to plasma A...


...ASMIC score or French score predicts the like...


...ested diagnostic and management str...


...re 2. A suggested diagnostic and manag...


Treatment

...Treatment...

...Immune-mediated TTP (...

...th iTTP experiencing a first acute event, the pane...

For patients with iTTP experiencing their first a...

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

...th iTTP experiencing a relapse, th...

...r patients with iTTP experiencing an acute e...

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


...Hereditary o...

...h cTTP who are in remission, the panel...

...tients with cTTP who are in remission,...

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

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

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


...ENTThe Guideline panel discussed a number...


...SECTION I. Supp...

...e following statements pertain to init...

...Statement...

...linicians generally consider a diagnos...

...Statement 2

... We did not systematically searc...

...Statement 3...

...x of suspicion for TTP is high, clinicians should...

...Stateme...

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

...Statem...

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

...Statement 6

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

...Statement 7...

...isk of catheter related complications such...

...Stateme...

... We did not systematically search...

...Statement 9...

...sed on indirect evidence in other critica...

...Sta...

...systematically search and review th...

...Statemen...

...did not systematically search and...


...SECTION II. Rel...

...In this section, the panel provides statemen...

...St...

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

...Statement 13...

...not systematically search and review...


...SECTION III. Other trea...

... The following nine statements pertain to the...

...Statement 14...

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

...Stateme...

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

...Statement...

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

...Statement 17...

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

...Statement 18

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

...Statement 19...

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

...Statement...

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

...Statement 21...

... We did not systematically search a...

...S...

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


...SECTION IV. TTP and...

...lowing statements pertain to women's health i...

...Statement 2...

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

...Stateme...

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

...Stateme...

...tients treated with rituximab are usua...

...Statement 26...

...men with a history of either cTTP or iTTP are us...

...Statement...

...TTP presenting in pregnancy generally merits trans...

Statement 28

...history of iTTP require close monitoring by a...

...Statemen...

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

...Statement 30...

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

...Statement 31...

... We did not systematically search an...

...Statement 32...

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


...SECTION V. Refusal o...

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

...S...

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