What is aTTP/iTTP?

Acquired/immune-mediated thrombotic thrombocytopenic purpura (aTTP/iTTP) is a rare, life-threatening, thrombotic microangiopathy manifested as microvascular thrombi and consequent thrombocytopenia, hemolytic anemia, and organ ischemia.2 Thrombotic microangiopathies (TMAs), such as aTTP/iTTP, can be hard to differentiate.6,7

Learn more about the different TMAs and how to distinguish one from another.

Types of TTP8-10

Acquired/immune-mediated TTP

  • The most common form of TTP; accounts for 95% of TTP cases
  • Caused by autoantibody inhibition of ADAMTS13 activity

Hereditary TTP

(also known as congenital TTP, inherited TTP, familial TTP, or Upshaw-Schulman syndrome)

  • Rare form of TTP
  • Caused by mutations in the ADAMTS13 gene

≈95% of TTP cases are aTTP/iTTP8

Each aTTP/iTTP episode can be unpredictable with microthrombi-driven risks5,11,12

The pathophysiology of aTTP/iTTP poses a triple threat; PEX and immunosuppressive therapy directly address only 2 of the 3 aspects of the disease9,13-18

Mechanism of disease Immunosuppressive therapy (eg, steroids): Autoantibody formation PEX: Autoantibody surplus and ADAMTS13 inhibition resulting in uncleaved vWF Unaddressed: Microthrombi-driven risk caused by platelet aggregation on ULvWF

Patient risk may remain in aTTP/iTTP despite treatment with PEX and immunosuppressive therapy4

8% to 20% of patients

Mortality remains high

8% to 20% of patients die despite receiving PEX and immunosuppressive therapy, according to a range of studies.1,2,4*

*Literature review of studies with more than 100 patients with TTP.

≈35% of deaths

Thromboembolic events caused by ischemia are common

Nearly 35% of in-hospital TTP deaths (613) were related to ischemia, including MI and stroke, despite receiving PEX.4†

†Retrospective claims analysis of hospitalizations with TTP (N=8203).

Up to 50% of patients

Relapse threatens patients

Up to 50% of patients have ≥1 recurrence within 30 days of stopping PEX.1‡

‡Retrospective review of French Reference Centre for TMA registry (N=388).

27.6% of patients

Increased risk of stroke through clinical remission§

Stroke after recovery with PEX occurred in 0% (0/22) of patients with mean normal ADAMTS13 activity (>70%) and in 27.6% (8/29) of patients with ADAMTS13 activity ≤70% (P=0.007).19‖

‖Cohort study of 170 patients with aTTP/iTTP from 1995 to 2018.

Warning symbol

Early diagnosis of aTTP/iTTP is critical

CABLIVI (caplacizumab-yhdp) dots

CABLIVI is targeted to prevent microthrombi

§Defined as sustained clinical response either with no PEX and no anti-vWF therapy in the last 30 days or with the attainment of ADAMTS13 remission (partial [between 20% and LLN] or complete [≥LLN]), whichever occurs first.20

ADAMTS13=a disintegrin and metalloproteinase with a thrombospondin type 1 motif, 13; LLN=lower limit of normal; MI=myocardial infarction; PEX=plasma exchange; TMA=thrombotic microangiopathy; TTP=thrombotic thrombocytopenic purpura; ULvWF=ultra-large von Willebrand factor; vWF=von Willebrand factor.

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