Table 1.

Main patient characteristics, episode clinical manifestations, and laboratory parameters.

Variables	N = 26
First TTP episode, n (%)	15 (57.7)
Follow-up, (d) , median (IQR)	279 (121-339)
Age, (y), median (IQR)	53 (47.3-60.4)
Female, n (%)	20 (76.9)
White ethnicity, n (%)	24 (92.3)
Platelet count/μL, median (IQR)	14,000 (10,250-18,750)
Hemoglobin, g/dL, median (IQR)a	9 (7.8-10.8)
Creatinine, mg/dL, median (IQR)b	1.08 (0.89-1.31)
LDH, IU/L, median (IQR)c	1243 (490-1919)
Total bilirubin, mg/dL, median (IQR)b	2.2 (1.6-3.5)
Indirect bilirubin, mg/dL, median (IQR)d	1.6 (1.3-2.4)
Systemic signs/symptoms, n (%)	22 (84.6)
Neurologic signs/symptoms, n (%)	15 (57.7)
Hemorrhagic signs/symptoms, n (%)	12 (46.2)
Cardiovascular signs/symptoms, n (%)	5 (19.2)
Renal signs/symptoms, n (%)	4 (15.4)

The median follow-up period was defined from the date of initiation of caplacizumab to the last documented follow-up. All clinical manifestations and laboratory parameters were recorded at the time of the acute TTP episode onset. Laboratory examinations were performed before plasma infusion or TPE. Clinical manifestations were categorized as follows:

• •
  systemic signs/symptoms: fatigue, fever, abdominal pain, headache, vomiting, jaundice (defined as total bilirubin levels ≥2.5 mg/dL);
• •
  neurologic signs/symptoms: ischemic stroke, transient ischemic attack, epileptic seizures, coma, personality disorders, focal neurologic signs;
• •
  hemorrhagic signs/symptoms: skin bleeding (purpura, bruising), mucosal bleeds (including epistaxis), hematuria, meno-metrorrhagia, gastrointestinal hemorrhages;
• •
  cardiovascular signs/symptoms: acute coronary syndrome, myocardial infarction, electrocardiographic ischemic changes;
• •
  renal signs/symptoms: acute renal failure, anuria, need for dialysis, hemoglobinuria, proteinuria, oliguria.

LDH, lactate dehydrogenase.

^aAvailable in 25 patients;

^bavailable in 23 patients;

^cavailable in 24 patients;

^davailable in 19 patients.