LFT, liver function test; n/a, not applicable.

Some patients were reported to have >1 adverse event. There were 17 bleeding episodes in 15 patients and 14 nonbleeding episodes in 11 patients.

Major bleeding was defined as per the International Society on Thrombosis Haemostasis bleeding scale guideline. Major bleeding in nonsurgical patients includes fatal bleeding and/or symptomatic bleeding in a critical area or organ (eg, intracranial, intraspinal, intraocular, retroperitoneal, intra-articular, or pericardial, or intramuscular with compartment syndrome) and/or bleeding causing a decrease in hemoglobin levels ≥2 g/dL or leading to transfusion of ≥2 units of whole blood or red cells.

This case was associated with a significant reduction in hemoglobin leading to drug interruption.

One case of severe upper gastrointestinal bleeding with subsequent decrease in hemoglobin on background of Barrett’s esophagus, gastroscopy showing small ulcer and esophagitis. One case of minor upper gastrointestinal bleeding.

One case of secondary hemorrhage of cerebral infarct and 1 case of intracranial hemorrhage following initial ischemic insult. There were no cases of primary intracerebral hemorrhage while on caplacizumab.

Postmechanical fall resulting in fractured femoral neck.

Thrombotic episodes included left brachial and right internal jugular thrombosis (n = 1) and pulmonary embolism (n = 4). These 5 episodes occurred in 4 patients. Two of these episodes occurred outside of the time during which the patient was being administered caplacizumab.

Caplacizumab was interrupted because of concerns managing bleeding risk on concurrent therapeutic anticoagulation in all cases.

One case had concurrent thrombosis.

Not listed on drug summary of product characteristics as a side effect.