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

Autor:	Uriol-Rivera MG; Nephrology Department, Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain.; Fundació Institut d'Investigació Sanitària Illes Balears (IdISBa), Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain., Andrade BL; Fundació Institut d'Investigació Sanitària Illes Balears (IdISBa), Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain.; Hematology Department, Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain., Bonet AM; Radiology Department, Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain., Mulet AO; Nephrology Department, Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain.; Fundació Institut d'Investigació Sanitària Illes Balears (IdISBa), Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain., Ruiz CB; Fundació Institut d'Investigació Sanitària Illes Balears (IdISBa), Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain.; Hematology Department, Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain., Parraga LP; Fundació Institut d'Investigació Sanitària Illes Balears (IdISBa), Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain.; Pharmacy Department, Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain., Lumbreras J; Fundació Institut d'Investigació Sanitària Illes Balears (IdISBa), Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain.; Pediatric Nephrology Department, Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain., Rota JIA; Fundació Institut d'Investigació Sanitària Illes Balears (IdISBa), Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain.; Intensive Care Unit, Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain., Servalos MF; Intensive Care Unit, Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain., Balaguer JF; Fundació Institut d'Investigació Sanitària Illes Balears (IdISBa), Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain.; Immunology Department, Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain., Ferreres LP; Internal Medicine Department, Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain., Valles MJP; Radiology Department, Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain., Valero RMRG; Gynecology and Obstetrics Department, Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain., Sanchez ST; Neurology Department, Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain., Martin AG; Neurology Department, Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain., Garcia JR; Preventive Medicine Department, Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain., Cobo CG; Fundació Institut d'Investigació Sanitària Illes Balears (IdISBa), Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain.; Clinical Analysis, Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain., Ramis-Cabrer D; Fundació Institut d'Investigació Sanitària Illes Balears (IdISBa), Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain.
Jazyk:	angličtina
Zdroj:	European journal of haematology [Eur J Haematol] 2024 Oct; Vol. 113 (4), pp. 510-520. Date of Electronic Publication: 2024 Jul 02.
DOI:	10.1111/ejh.14261
Abstrakt:	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 a severe ADAMTS-13 deficiency (defined as a disintegrin-like and metalloprotease with thrombospondin type 1 motif no. 13 activity ≥10%: TMA-13n) continue to experience elevated mortality rates. This study explores the prognostic indicators for augmented mortality risk or necessitating chronic renal replacement therapy (composite outcome: CO) in TMA-13n patients. We included 42 TMA-13n patients from January 2008 to May 2018. Median age of 41 years and 60% were female. At presentation, 62% required dialysis, and 57% warranted intensive care unit admission. CO was observed in 45% of patients, including a 9-patient mortality subset. Multivariate logistic regression revealed three independent prognostic factors for CO: early administration of eculizumab (median time from hospitalization to eculizumab initiation: 5 days, range 0-19 days; odds ratio [OR], 0.14; 95% confidence interval [CI], 0.02-0.94), presence of neuroradiological lesions (OR, 6.67; 95% CI, 1.12-39.80), and a PLASMIC score ≤4 (OR, 7.39; 95% CI, 1.18-46.11). In conclusion, TMA-13n patients exhibit a heightened risk of CO in the presence of low PLASMIC scores and neuroradiological lesions, while early eculizumab therapy was the only protective factor. (© 2024 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)
Databáze:	MEDLINE
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