History of the patient, ADAMTS13 activity, and inhibitors and laboratory parameters. (A) Timeline history of the patient. Treatments: plasma exchange (▾); immunoglobulins (Ig); corticosteroids (circles with vertical lines); fresh frozen plasma (FFP); vincristine (Vin); antiplatelet agents (ATG); cyclosporine A (CyA); cyclophosphamide (CHP); plasma exchange, vincristine, antiplatelet agents, and corticosteroids (#). Filled rectangle indicates splenectomy; ↓, TIA; filled down arrow, ADAMTS13 activity less than 6% (normal range, 50%-150%), ADAMTS13 inhibitors present; and (), platelet count. (B) ADAMTS13 activity (□) and anti-ADAMTS13 inhibitor titer (○) in the 2 courses of plasma exchange (▾), during rituximab (▵) treatment and in the follow-up period. § indicates IgG titer, 1:1600; IgM, negative. : IgG titer, negative; IgM, negative. The upward-pointing filled arrowhead indicates time of sampling for immunoblot (see Figure 2A); and the vertical double-headed arrow, time of sampling for VWF multimer analysis (see Figure 2B). (C) Platelet count (; normal range, 140 × 10⁹/L-440 × 10⁹/L), LDH (▪; normal range, 240 U/L-460 U/L), and haptoglobin (•; normal range, 49 mg/dL-246 mg/dL). (D) Lymphocytes count (⊕; normal range, 1.000-5.000 × 10⁶/L) and CD20 count (⋄; normal range, 5%-15% of lymphocytes count).