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
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.
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).
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
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:
Characterized by the presence of schistocytes
Lesions can occur in any organ, but they frequently affect the heart, brain, and kidney
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.
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.
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.