SummaryThrombotic thrombocytopenic purpura (TTP) is characterised by the systemic microvascular aggregation of platelets causing ischaemia of the brain and other organs. We describe the case of a 54 year-old man who presented with neurological signs, fever, severe thrombocytopenia, microangiopathic haemolytic anaemia and renal failure 5 days after receiving an influenza vaccination. He was diagnosed with acute refractory TTP caused by autoantibody-mediated ADAMTS-13 deficiency. He required stabilisation on the critical care unit before being successfully treated with 3 l plasma exchanges for 21 days and rituximab (MabThera

Introduction: Medium cutoff (MCO) membranes for hemodialysis (HD) remove more effectively large middle molecules than high-flux (HF) membranes. In patients on in-center short frequent HD regimen (5 sessions per week, 2 hours and 30 minutes per session) the effect of MCO on middle weight uremic toxins has not been elucidated. Methods: This retrospective study included 15 patients previously performing short frequent HD with HF dialyzer (HF-HD), that were switched to short frequent HD with MCO dialyzer (MCO-HD) for 2 months, and returned to HF-HD. The primary endpoint was the predialysis concentration of α1-acid glycoprotein during the different study phases. Secondary endpoints were predialysis concentration of other middle molecules, albumin, and assessment of the quality of life using the 36-item short-form health survey (SF-36). Findings: During MCO-HD phase there was a reduction in mean ± SD α1-acid glycoprotein concentration (98.71 ± 25.2 vs. 88.6 ± 24.6 mg/dL, P = 0.107), followed by an increment 2 months after returning to HF-HD (89.18 ± 26.12 vs. 97.33 ± 31.29 mg/dL, P = 0.002); however, only the second variation was statistically significant. MCO-HD provided lower median predialysis concentration of prolactin (16 [10.2-25.6] vs. 14.1 [11.7-34.8] ng/mL, P = 0.036). Single-pool Kt/V, standard Kt/V, predialysis β2-microglobulin, myoglobin, and SF-36 questionnaire remained stable during the first two phases (pre-MCO and MCO). β2-Microglobulin increased in the post-MCO phase (20.02 ± 8.14 vs. 21.27 ± 7.64 μg/mL, P = 0.000). Mean predialysis concentration of albumin reduced significantly from pre-MCO vs. MCO phases (39.9 ± 3.7 vs. 38.3 ± 3.3 g/L, P = 0.020

Rationale: Protocols for regional citrate anticoagulation with the hypertonic 4% trisodium citrate solution have been recently described as an anticoagulation strategy during membrane therapeutic plasma exchange (mTPE). The effect of citrate in the patient’s systemic hemostasis is negligible, thus regional citrate anticoagulation application is advantageous in circumstances in which heparin-based protocols are deemed unsafe for patients with a high risk of bleeding. The downsides of using hypertonic citrate solutions are mainly hypocalcemia and hypernatremia that ultimately can cause adverse clinical events. Presenting concerns of the patient: (1) A 57-year-old Caucasian female with a history of active vaginal bleeding secondary to endometrial hyperplasia. She had a history of antiphospholipid syndrome, and systemic lupus erythematosus with marked refractory autoimmune thrombocytopenia. Her platelet count was persistently below 4,000/mm3 even after different immunosuppressive regimens and daily platelet transfusions. (1) A 70-year-old Caucasian female was hospitalized presenting acute kidney injury stage 3 due to rapidly progressive antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, however without the need for renal replacement therapy. At admission, serum creatinine (sCr) was 3.56 mg/dL (normal range: 0.53-1.00 mg/dL). Her baseline sCr was 0.8 mg/dL obtained 6 months earlier. Chest tomography revealed bilateral masses compatible with granulomatous lesions and no signs of alveolar bleeding. Since severe cases of ANCA vasculitis involving the lungs may evolve with alveolar hemorrhage, heparin was avoided. Diagnoses: (1) Systemic lupus erythematosus-associated autoimmune thrombocytopenia and (2) ANCA-associated vasculitis with kidney and lung involvement. Interventions: Herein, we describe a case series of 12 consecutive mTPE treatments in 2 different patients using regional 4% trisodium citrate anticoagulation. Outcomes: All the sessions were uneventful, presented only minor electrolyte imbalances, and were effectively completed without early interruptions due to clotting of the plasmafilter. Teaching points: In our 2 cases, extracorporeal regional citrate anticoagulation was successful in optimizing plasmafilter patency without bleeding events in 2 high-risk patients using established protocols for the citrate and calcium infusions.