More about low-dose rituximab and plasma exchange as front-line therapy for patients with thrombotic thrombocytopenic purpura

Introduction: Thrombotic thrombocytopenic purpura (TTP) is characterized by a reduction in the von Willebrand cleavage protein ADAMTS-13, mainly as a consequence of autoimmunity. Plasma exchange (PEx) is standard, achieving complete remission (CR) in 77–83% of cases, but rates are variable depending on ADAMTS-13 activity and relapse is frequent in patients with <10%. Thus, an effective front-line immunosuppressive treatment is needed.Materials and methods: We administered PEx daily until CR and rituximab 100 mg/dose/week for 4 consecutive weeks to 10 patients with a first TTP episode and 1 relapsed patient (8 females (72%) and 3 males (28%)). Median age was 34 years (15–46) and laboratory parameters at diagnosis were as follows: platelets 11 × 109/l (range 7–27.4 × 109/l), lactate dehydrogenase 1822 U/l (range 705–8220 U/l, normal 70–180 U/l), and haemoglobin 6 g/dl (range 4.2–11.8 g/dl). ADAMTS-13 activity was determined in eight patients and was <10% in all. ADAMTS-13 autoantibody titre was determined i...