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aTTP is a rare, rapidly progressing, life-threatening, relapsing autoimmune disease1-5

Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare, life-threatening thrombotic microangiopathy (TMA) that is characterized by hemolytic anemia, severe thrombocytopenia, and organ ischemia. Before plasma exchange was adopted in clinical practice, roughly 90% of patients presenting with acute TTP died.2

Danger sign

Mortality remains high

8% to 20% of patients die despite receiving PEX and immunosuppressive therapy, according to a range of registries1-3*

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


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 PEX3†

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


Risk persists post-discharge

Approximately 50% of patients have a recurrence within 30 days of stopping PEX1‡

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

TTP can be either inherited or acquired. 95% of all TTP is acquired.5

Risk remains in aTTP despite current treatment with PEX and immunosuppressive therapy2,4

Complex presentation of aTTP often masks its diagnosis

As a result of its underlying pathology, aTTP presents with highly variable, multiorgan symptoms that most commonly include, but are not limited to purpura, petechiae, epistaxis, headache, confusion, dyspnea, stroke, and MI.5 aTTP should be suspected based on the following:


Severe thrombocytopenia2

Platelets <30x109/L


Microangiopathic hemolytic anemia (MAHA)2

Characterized by the presence of schistocytes

Heart Brain and Kidney

Organ ischemia2

Lesions can occur in any organ, but they frequently affect the heart, brain, and kidney

Danger sign

aTTP poses dangerous risks


CABLIVI can help make a difference

MI=myocardial infarction; PEX=plasma exchange; TMA=thrombotic microangiopathy; TTP=thrombotic thrombocytopenic purpura.



CABLIVI is contraindicated in patients with a previous severe hypersensitivity reaction to caplacizumab-yhdp or to any of its excipients. Hypersensitivity reactions have included urticaria.


Bleeding Risk:

  • CABLIVI increases the risk of bleeding. In clinical studies, severe bleeding adverse reactions of epistaxis, gingival bleeding, upper gastrointestinal hemorrhage, and metrorrhagia were each reported in 1% of subjects. Overall, bleeding events occurred in approximately 58% of patients on CABLIVI versus 43% of patients on placebo. The risk of bleeding is increased, in patients with underlying coagulopathies and concomitant use of CABLIVI with drugs affecting hemostasis.
  • If clinically significant bleeding occurs, interrupt use of CABLIVI. Von Willebrand factor concentrate may be administered to rapidly correct hemostasis. If CABLIVI is restarted, monitor closely for signs of bleeding.
  • Withhold CABLIVI for 7 days prior to elective surgery, dental procedures or other invasive interventions. If emergency surgery is needed, the use of von Willebrand factor concentrate may be considered to correct hemostasis. After the risk of surgical bleeding has resolved, and CABLIVI is resumed, monitor closely for signs of bleeding.


The most common adverse reactions (>15% of patients) were epistaxis (29%), headache (21%) and gingival bleeding (16%).


Concomitant use of CABLIVI with any anticoagulant may increase the risk of bleeding. Assess and monitor closely for bleeding with concomitant use.


There are no available data on CABLIVI use in pregnant women to inform a drug associated risk of major birth defects and miscarriage.

  • Fetal/neonatal adverse reactions: CABLIVI may increase the risk of bleeding in the fetus and neonate. Monitor neonates for bleeding.
  • Maternal adverse reactions: All patients receiving CABLIVI, including pregnant women, are at risk for bleeding. Pregnant women receiving CABLIVI should be carefully monitored for evidence of excessive bleeding.


CABLIVI (caplacizumab-yhdp) is indicated for the treatment of adult patients with acquired thrombotic thrombocytopenic purpura (aTTP), in combination with plasma exchange and immunosuppressive therapy.