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CME: Congenital Thrombotic Thrombocytopenic Purpura (cTTP): Updates from the American Society of Hematology (ASH) 2019 annual meeting


Activity Description / Statement of Need:

In this online, self-learning activity:

Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder that impacts three in a million adults per year, with congenital or hereditary TTP (cTTP, also known as Upshaw-Schulman syndrome) accounting for a third of the overall incidence. The incidence of TTP rises with increasing age and the mortality rate of untreated TTP may be as high as 90%.

In recent years molecular mechanisms contributing to TTP have been identified: patients diagnosed with TTP have larger von Willebrand factor molecules (vWF) and a defective protease enzyme of A Disintegrinlike And Metalloprotease with ThromboSpondin type 1 motif 13 (ADAMTS13), which cleaves larger vWF molecules and inhibits platelet adhesion. In congenital or hereditary TTP (cTTP, also known as Upshaw-Schulman syndrome), the gene that codes of ADAMTS13 is defective and cannot properly produce the enzyme, whereas in acquired TTP, antibody production leads to downstream enzymatic deactivation.

Target Audience:

The following healthcare professionals: hematologists; physician assistants, nurse practitioners, nurses, and pharmacists who practice in hematology; and any other healthcare professionals with an interest in or who clinically encounter patients with cTTP.

This program is supported by an educational grant from Takeda / Shire.

Release Date: April 30, 2020 -- Expiration Date: April 30, 2022

Faculty: Satheesh Chonat, MD


Pathophysiology of TTP

Diagnosis of TTP

Clinical Manifestations or Diagnostic Opportunities

Highlights of Research Presented at ASH 2019

Learning Objectives

By the end of the session the participant will be able to:

  • Summarize the most impactful findings presented at the ASH 2019 annual meeting relating to congenital TTP
  • Recall the pathophysiology of congenital TTP
  • Describe the clinical manifestations and methods of establishing a diagnosis of cTTP, and apply that information to a patient case
  • List treatment strategies for cTTP, and apply that information to a patient case


ACCME Activity #201861263


Faculty Disclosure and Resolution of COI

As a provider of continuing medical education, it is the policy of ScientiaCME to ensure balance, independence, objectivity, and scientific rigor in all of its educational activities. In accordance with this policy, faculty and educational planners must disclose any significant relationships with commercial interests whose products or devices may be mentioned in faculty presentations, and any relationships with the commercial supporter of the activity. The intent of this disclosure is to provide the intended audience with information on which they can make their own judgments. Additionally, in the event a conflict of interest (COI) does exist, it is the policy of ScientiaCME to ensure that the COI is resolved in order to ensure the integrity of the CME activity. For this CME activity, any COI has been resolved thru content review ScientiaCME.

Faculty Disclosure: Satheesh Chonat M.D. , Assistant Professor, Emory University, has received financial compensation from Agios, Alexion, Amgen, and Global Therapeutics (Grants and /or Clinical Trial Funding).

Disclosures of Educational Planners: Charles Turck, PharmD has no relevent financial disclosures.

Commercial Support Disclosure: This program is supported by an educational grant from Takeda / Shire.


  • Read the learning objectives above
  • Take the Pre-Test (optional). Completion of the pre-test will help us evaluate the knowledge gained by participating in this CME activity.
  • View the online activity. You may view this is in more than one session, and may pause or repeat any portion of the presentation if you need to.
  • Minimum participation threshold: Take the post-test. A score of 70% or higher is required to pass and proceed to the activity evaluation.
  • Complete the activity evaluation and CME registration. A CE certificate will be emailed to you immediately.

Cultural/Linguistic Competence & Health Disparities

System Requirements

Windows 7 or above
Internet Explorer 8
*Adobe Acrobat Reader
Mac OS 10.2.8
Safari or Chrome or Firefox
*Adobe Acrobat Reader
Internet Explorer is not supported on the Macintosh

*Required to view Printable PDF Version

Perform Pre-Test (optional)

Please take a few minutes to participate in the optional pre-test. It will help us measure the knowledge gained by participating in this activity.

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