Predictive Factors of Mortality in Population of Patients with Paroxysmal Nocturnal Hemoglobinuria (PNH): Results from a Korean PNH Registry

Journal of Korean Medical Science
Jun Ho JangJong Wook Lee

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is a progressive, systemic, life-threatening disease, characterized by chronic uncontrolled complement activation. A retrospective analysis of 301 Korean PNH patients who had not received eculizumab was performed to systematically identify the clinical symptoms and signs predictive of mortality. PNH patients with hemolysis (lactate dehydrogenase [LDH] ≥ 1.5 × the upper limit of normal [ULN]) have a 4.8-fold higher mortality rate compared with the age- and sex-matched general population (P < 0.001). In contrast, patients with LDH < 1.5 × ULN have a similar mortality rate as the general population (P = 0.824). Thromboembolism (TE) (odds ratio [OR] 7.11; 95% confidence interval [CI] (3.052-16.562), renal impairment (OR, 2.953; 95% CI, 1.116-7.818) and PNH-cytopenia (OR, 2.547; 95% CI, 1.159-5.597) are independent risk factors for mortality, with mortality rates 14-fold (P < 0.001), 8-fold (P < 0.001), and 6.2-fold (P < 0.001) greater than that of the age- and sex-matched general population, respectively. The combination of hemolysis and 1 or more of the clinical symptoms such as abdominal pain, chest pain, or dyspnea, resulted in a much greater increased mortality rate when compared with p...Continue Reading

References

Nov 9, 1995·The New England Journal of Medicine·P HillmenJ V Dacie
Feb 6, 2004·The New England Journal of Medicine·Peter HillmenRussell P Rother
Jul 30, 2005·Blood·Charles ParkerUNKNOWN International PNH Interest Group
Sep 23, 2006·The New England Journal of Medicine·Peter HillmenLucio Luzzatto
Jun 7, 2008·Blood·Régis Peffault de LatourUNKNOWN French Association of Young Hematologists
Apr 24, 2013·Blood·Anita HillPeter Hillmen

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Citations

Oct 26, 2016·International Journal of Hematology·Haruhiko NinomiyaYuzuru Kanakura
Oct 22, 2016·Thrombosis Journal·Wen-Chien ChouUNKNOWN Taiwan PNH study group
Sep 2, 2018·European Journal of Haematology·Timothy DevosBérangère Devalet
Dec 5, 2017·Nature Reviews. Nephrology·Daniel RicklinJohn D Lambris
Feb 6, 2020·Expert Opinion on Biological Therapy·Jong Wook Lee, Austin G Kulasekararaj
Dec 21, 2018·Revista da Associação Médica Brasileira·Ênio Simas MacedoElizabeth De Francesco Daher
Jul 1, 2019·Annals of Hematology·Jin Seok KimUNKNOWN Aplastic Anemia Working Party of the Korean Society of Hematology
Dec 1, 2020·Expert Opinion on Drug Safety·Sung-Eun Lee, Jong Wook Lee
Sep 17, 2021·Orphanet Journal of Rare Diseases·Carolyn E SchwartzKarl-Johan Myren

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Methods Mentioned

BETA
flow cytometry
MDS

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