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Clinical implication of renal dysfunction during the clinical course in patients with paroxysmal nocturnal hemoglobinuria: a longitudinal analysis

Title
Clinical implication of renal dysfunction during the clinical course in patients with paroxysmal nocturnal hemoglobinuria: a longitudinal analysis
Authors
Kim, Jin SeokCheong, June-WonMun, Yeung-ChulJang, Jun HoJo, Deog-YeonLee, Jong WookKorean Soc Hematology
Ewha Authors
문영철
SCOPUS Author ID
문영철scopus
Issue Date
2019
Journal Title
ANNALS OF HEMATOLOGY
ISSN
0939-5555JCR Link

1432-0584JCR Link
Citation
ANNALS OF HEMATOLOGY vol. 98, no. 10, pp. 2273 - 2281
Keywords
Paroxysmal nocturnal hemoglobinuriaRenal dysfunctionThromboembolismMortality
Publisher
SPRINGER
Indexed
SCI; SCIE; SCOPUS WOS scopus
Document Type
Article
Abstract
Although renal dysfunction at the time of diagnosis of paroxysmal nocturnal hemoglobinuria (PNH) is a risk factor for mortality, subsequent renal events can occur. The objective of this study was to identify clinical implication of renal dysfunction occurring during the disease course in PNH patients. One hundred one patients with a granulocyte clone size of > 10% were enrolled. Renal events were observed in 55 (54.5%) patients during a median follow-up of 94.2 months. Median time to first renal event from diagnosis of PNH was 79.3 months. Thromboembolism (TE) event and recurrent TE events were observed in 25 (24.8%) and 8 (7.9%) patients, respectively. The rate of recurrent TE was significantly higher in patients with renal events >= 2 compared with that in patients with renal event <= 1 (18.8% vs. 2.9%; P = 0.012). The rate of recurrent TE was significantly higher in patients with chronic kidney disease (CKD) + acute kidney disease (AKD) compared with the rest of the patients (27.3% vs. 5.6%; P = 0.040). CKD+AKD was the only independent risk factor for OS in multivariate analysis (hazard ratio 7.95, 95% CI 1.24-51.15, P = 0.029). Therefore, close monitoring of renal events in PNH patients during the entire clinical course is essential.
DOI
10.1007/s00277-019-03735-6
Appears in Collections:
의과대학 > 의학과 > Journal papers
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