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Clinical Implication of Renal Dysfunction During the Clinical Course in Patients With Paroxysmal Nocturnal Hemoglobinuria: A Longitudinal Analysis

Authors
 Jin Seok Kim  ;  June-Won Cheong  ;  Yeung-Chul Mun  ;  Jun Ho Jang  ;  Deog-Yeon Jo  ;  Jong Wook Lee  ;  Aplastic Anemia Working Party of the Korean Society of Hematology 
Citation
 ANNALS OF HEMATOLOGY, Vol.98(10) : 2273-2281, 2019-10 
Journal Title
 ANNALS OF HEMATOLOGY 
ISSN
 0939-5555 
Issue Date
2019-10
MeSH
Acute Kidney Injury* / epidemiology ; Acute Kidney Injury* / etiology ; Adolescent ; Adult ; Aged ; Aged, 80 and over ; Child ; Female ; Follow-Up Studies ; Hemoglobinuria, Paroxysmal* / complications ; Hemoglobinuria, Paroxysmal* / epidemiology ; Humans ; Longitudinal Studies ; Male ; Middle Aged ; Renal Insufficiency, Chronic* / epidemiology ; Renal Insufficiency, Chronic* / etiology ; Thromboembolism / epidemiology ; Thromboembolism / etiology
Keywords
Mortality ; Paroxysmal nocturnal hemoglobinuria ; Renal dysfunction ; Thromboembolism
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.
Full Text
https://link.springer.com/article/10.1007%2Fs00277-019-03735-6
DOI
10.1007/s00277-019-03735-6
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Jin Seok(김진석) ORCID logo https://orcid.org/0000-0001-8986-8436
Cheong, June-Won(정준원) ORCID logo https://orcid.org/0000-0002-1744-0921
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/175808
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