case_images: PMC11371688_01
case_images: Summary of laboratory parameters and treatment administered in our case. TPE daily, days 0-4 and 6-15, temporarily increased bid days 9-12; intravenous methylprednisolone 1.5 mg/kg daily, days 0-16 temporarily increased to pulse 15 mg/kg/day, days 9-12; oral prednisone 1 mg/kg then slowly tapered, days 13-51; intravenous rituximab, 375 mg/m2, on days 7, 10, 15, and 20; caplacizumab 5 mg daily, intravenous day 12 and subcutaneous days 13-54, and then every second day, days 55-64. The functional ADAMTS13 inhibitor level is semiquantitative when the value is >2 BU/ml, >>2 BU/ml, being the highest. TPE, therapeutic plasma exchange; IV, intravenous; G/L, giga/liter; BU/ml, Bethesda unit/milliliter. ADAMTS13 activity normal range, 51%-100%. Functional ADAMTS13 inhibitor normal range, <0.4 BU/ml.
case_images: fped-12-1448801-g001.jpg
case_images: PMC11371688_01_fped-12-1448801-g001.jpg
case_images: F1
case_images: However, this was rapidly followed by an iTTP exacerbation, with the platelet count dropping to 40 G/L on D6 (Figure 1), increased hemolysis, and signs of cardiac and renal injury. Her platelet count increased to 140 G/L after 2 days and remained in the normal range thereafter (Figure 1).