Risk factors of death or chronic renal replacement therapy requirements in patients with thrombotic microangiopathies without ADAMTS‐13 deficiency

• DOI: 10.1111/ejh.14261
• Publication Date: 2024-07-03T02:44:03Z
• Authors: Miguel G. Uriol‐R...; Bernardo López An...; Antonio Mas Bonet; Aina Obrador Mulet; Carmen Ballester ...; Leonor Periañez P...; Javier Lumbreras; José Ignacio Ayes...; Mireia Ferreruela...; Joana Ferrer Bala...; Lucio Pallares Fe...; María Jose Picado...; Rosa María Ruíz d...; Susana Tarongi Sa...; Ana Garcia Martin; Juan Rodríguez Ga...; Cristina Gomez Cobo; Daniel Ramis‐Cabrer
• Keywords: Renal replacement therapy; ADAMTS

Thrombotic microangiopathy (TMA), characterized by microangiopathic hemolytic anemia, thrombocytopenia, and multisystem organ dysfunction, is a life-threatening disease. Patients with TMA who do not exhibit severe ADAMTS-13 deficiency (defined as disintegrin-like metalloprotease thrombospondin type 1 motif no. 13 activity ≥10%: TMA-13n) continue to experience elevated mortality rates. This study explores the prognostic indicators for augmented risk or necessitating chronic renal replacement therapy (composite outcome: CO) in TMA-13n patients. We included 42 patients from January 2008 May 2018. Median age of 41 years 60% were female. At presentation, 62% required dialysis, 57% warranted intensive care unit admission. CO was observed 45% patients, including 9-patient subset. Multivariate logistic regression revealed three independent factors CO: early administration eculizumab (median time hospitalization initiation: 5 days, range 0-19 days; odds ratio [OR], 0.14; 95% confidence interval [CI], 0.02-0.94), presence neuroradiological lesions (OR, 6.67; CI, 1.12-39.80), PLASMIC score ≤4 7.39; 1.18-46.11). In conclusion, heightened low scores lesions, while only protective factor.