#3479 RISK FACTORS OF DEATH OR CHRONIC RENAL REPLACEMENT THERAPY REQUIREMENTS IN PATIENTS WITH THROMBOTIC MICROANGIOPATHIES WITHOUT ADAMTS-13 DEFICIENCY

Abstract

Abstract Background and Aims Thrombotic microangiopathy without severe ADAMTS-13 deficiency activity levels (TMA-13n) has a high mortality rate. Early recognition of patients at higher risk of dying or needing chronic renal replacement therapy (composite outcome: CO) has become of paramount importance since treatments such as eculizumab could ameliorate the prognosis of this group of patients. Plasmic score have been shown to predict severe ADAMTS-13 deficiency; however, its role as a prognostic factor of CO in TMA-13n is unknown. Method We retrospectively evaluate the potential factors associated with the risk of CO. Patients diagnosed with TMA-13n (defined by microangiopathic anaemia, thrombocytopenia, organ damage and ADAMTS-13 activity ≥ 10%) from January 2008 to May 2018 in our centre were included in the study. Results Forty-two consecutive patients were included. Mean (SD) age: 41(20) years. Twenty-five (60% were females. Mean (SD) ADAMTS-13 activity: 69 (24%). Twenty-six (62%) patients required dialysis at admission. Nineteen (45%) met CO (10 patients died). Higher age, lower lactate dehydrogenase, PLASMIC score values ≤ 4, neuroradiological damage and early eculizumab use [median (IQR): 4 (0-16) days from hospital admission: 5] were factors associated with the risk for developing CO. Only three factors were independently associated with CO mortality by logistic regression: early use of eculizumab (OR: 0.14; 95% CI: 0.02-0.94) and neuroradiological damage (OR: 6.67; 95% CI: 1.12-39.80) and PLASMIC score ≤ 4 (OR: 7.39; 95% CI: 1.18-46.11). Conclusion In TMA-13n patients, neuroradiological damage, low PLASMIC score and early use of eculizumab were independent prognostic factors of death and chronic renal replacement therapy requirements.