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