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Title: Presence of acute and chronic renal failure in patients with paroxysmal nocturnal hemoglobinuria: results of a retrospective analysis from the Spanish PNH Registry
Authors: Villegas, Ana
Núñez, Ramiro
Gaya, Anna
Cuevas-Ruiz, María Victoria
Bosch, José Miguel 
Carral, Anna
Arrizabalaga, Beatriz
Gómez-Roncero, María Isabel
Mora, Asunción
Bravo, Pilar
Lavilla, Esperanza
Monteserín, Carmen
Hernández, Belén
Martínez-Barranco, Pilar
Jarque, Isidro
Urquía, María Anunciación
García-Donas, Gloria
Brunet, Salut
González, Fernando Ataulfo
Urbano, Álvaro
UNESCO Clasification: 32 Ciencias médicas
320504 Hematología
Keywords: Paroxysmal nocturnal hemoglobinuria (PNH)
Acute and chronic renal failure
Glomerular filtration rate
Renal function status
Issue Date: 2017
Journal: Annals of Hematology 
Abstract: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, life-threatening blood disease. With the advent of eculizumab treatment, renal function has substantially improved, although no data from real-world clinical practice are available. An observational, retrospective, multicenter study was conducted in Spain on clinical data obtained from outpatient visits of patients with PNH (Spanish PNH Registry) who had experienced acute (ARF) or chronic (CRF) renal failure. Of the 128 patients registered (April 2014), 60 were diagnosed with classic PNH. Twenty-seven (45.0%) patients with a mean age of 48.5 (±16.2) years had renal failure, ARF or CRF, and were included in this study. Near half of the patients (n = 13; 48.1%) presented with ARF alone, 33.3% (n = 9) had CRF with episodes of ARF, while 18.5% (n = 5) were diagnosed with CRF alone. For patients with diagnosis of PNH and renal failure (n = 27), the median time to the first ARF episode was 6.5 (CI 95%; 2.2, 14.9) years, whereas the median to the diagnosis of CRF was 14.5 (CI 95%; 3.8, 19.2) years after the diagnosis of PNH. Patients with ARF (n = 22) were treated with eculizumab and did not experience new episodes of ARF, except for one patient with sepsis. Of the patients with CRF, two received treatment without experiencing further episodes of ARF. Sixteen patients who completed treatment (11 with ARF and 5 with ARF + CRF) recovered from the episode of ARF or from CRF. Of the remaining patients treated with eculizumab, one patient improved from stages III to II, three patients stabilized without showing disease progression, and one patient progressed from stages III to IV. Treatment with eculizumab in PNH patients has beneficial effects on renal function, preventing ARF and progression to CRF.
ISSN: 0939-5555
DOI: 10.1007/s00277-017-3059-x
Source: Annals of Hematology[ISSN 0939-5555],v. 96, p. 1727-1733 (Julio 2017)
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