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Clinical signs and symptoms associated with increased risk for thrombosis in patients with paroxysmal nocturnal hemoglobinuria from a Korean Registry.

Authors
 Jong Wook Lee  ;  Jun Ho Jang  ;  Jin Seok Kim  ;  Sung-Soo Yoon  ;  Je-Hwan Lee  ;  Yeo-Kyeoung Kim  ;  Deog-Yeon Jo  ;  Jooseop Chung  ;  Sang Kyun Sohn 
Citation
 INTERNATIONAL JOURNAL OF HEMATOLOGY, Vol.97(6) : 749-757, 2013 
Journal Title
 INTERNATIONAL JOURNAL OF HEMATOLOGY 
ISSN
 0925-5710 
Issue Date
2013
MeSH
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Child ; Female ; Hemoglobinuria, Paroxysmal/complications* ; Hemoglobinuria, Paroxysmal/diagnosis ; Humans ; Lactate Dehydrogenases/metabolism ; Male ; Middle Aged ; Prognosis ; ROC Curve ; Registries ; Republic of Korea ; Retrospective Studies ; Risk Factors ; Thromboembolism/diagnosis ; Thromboembolism/etiology ; Thrombosis/diagnosis* ; Thrombosis/etiology* ; Young Adult
Keywords
Risk Factor ; Hemolysis ; Thrombosis ; Mortality ; Paroxysmal nocturnal hemoglobinuria
Abstract
Paroxysmal nocturnal hemoglobinuria (PNH) is characterized by chronic, complement-mediated hemolysis, frequently leading to debilitating clinical symptoms and life-threatening complications such as thromboembolism (TE). A retrospective analysis was performed on 301 patients from the South Korean National PNH Registry to describe disease burden and identify TE-associated risk factors. TE was identified in 18 % of patients and was associated with increased risk for mortality [odds ratio (OR), 6.85; P < 0.001]. A multivariate analysis showed that PNH patients with elevated hemolysis [lactate dehydrogenase (LDH) levels ≥1.5 times the upper limit of normal (ULN)] at diagnosis were at significantly higher risk for TE than patients with LDH <1.5 × ULN (OR 7.0; P = 0.013). The combination of LDH ≥1.5 × ULN with the clinical symptoms of abdominal pain, chest pain, dyspnea, or hemoglobinuria was associated with a greater increased risk for TE than elevated hemolysis or clinical symptoms alone. Continuous monitoring of these risk factors is critical for identifying PNH patients at risk for morbidities and mortality and allowing early intervention.
Full Text
https://link.springer.com/article/10.1007%2Fs12185-013-1346-4
DOI
10.1007/s12185-013-1346-4
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
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/165804
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