Prevention and Management of Thromboembolism in Patients with Paroxysmal Nocturnal Hemoglobinuria in Asia: A Narrative Review.
Journal
International journal of molecular sciences
Journal Volume
26
Journal Issue
6
ISSN
1422-0067
Date Issued
2025-03-11
Author(s)
Ueda, Yasutaka
Goh, Yeow-Tee
Rojnuckarin, Ponlapat
Kim, Jin Seok
Wong, Raymond Siu Ming
Lee Wong, Lily Lee
Jang, Jun Ho
Chiou, Tzeon-Jye
Kanakura, Yuzuru
Lee, Jong Wook
Abstract
Thromboembolism (TE) is a major cause of morbidity and mortality in patients with paroxysmal nocturnal hemoglobinuria (PNH). This narrative review summarizes available evidence on TE in Asian patients with PNH and discusses practical considerations and challenges for preventing and managing PNH-associated TE in Asian populations. Evidence suggests that, compared with non-Asians, fewer Asian patients have a history of TE (3.6% vs. 8.9%, < 0.01), receive anticoagulants (8.5% vs. 16.2%, = 0.002), or die from TE (6.9% vs. 43.7%, = 0.000). Independent predictors of TE include lactate dehydrogenase ≥ 1.5 × upper limit of normal, pain, and male sex. Clone size alone does not appear to be a reliable estimate of TE risk. D-dimer levels are a useful marker of hemostatic activation, although they are not specific to PNH. Complement inhibition reduces the incidence of TE, although it does not wholly eliminate TE risk. Eligibility criteria and access to complement inhibitors vary across Asia, with limited availability in some countries. Anticoagulation is required to treat acute TE events and for primary or secondary prophylaxis in selected patients. Physicians and patients must stay alert to the signs and symptoms of TE to ensure prompt and appropriate treatment.
Subjects
Asia
complement inhibitor
paroxysmal nocturnal hemoglobinuria
thromboembolism
thrombosis
Publisher
MDPI
Type
review