Monitoring and Treatment of Paroxysmal Nocturnal Hemoglobinuria in Patients with Aplastic Anemia in Asia: An Expert Consensus.
Journal
International journal of molecular sciences
Journal Volume
25
Journal Issue
22
ISSN
1422-0067
Date Issued
2024-11-13
Author(s)
Wong, Raymond Siu Ming
Ho Jang, Jun
Wong, Lily Lee Lee
Kim, Jin Seok
Rojnuckarin, Ponlapat
Goh, Yeow-Tee
Ueda, Yasutaka
Lee, Jong Wook
Kanakura, Yuzuru
Chiou, Tzeon-Jye
DOI
10.3390/ijms252212160
Abstract
Paroxysmal nocturnal hemoglobinuria (PNH) clones can be identified in a significant proportion of patients with aplastic anemia (AA). Screening for PNH clones at the time of an AA diagnosis is recommended by national and international guidelines. In this report, an expert panel of physicians discusses current best practices and provides recommendations for managing PNH in patients with AA in the Asia-Pacific region. Plasma/serum lactate dehydrogenase (LDH) levels and reticulocyte count should be measured with every blood test. PNH clone size should be monitored regularly by flow cytometry, with on-demand testing in the event of a rise in LDH level ± reticulocyte count or development of symptoms such as thrombosis. Monitoring for PNH clones can guide the choice of initial AA treatment, although flow cytometry has resource implications which may present a challenge in some Asia-Pacific countries. The treatment of patients with both PNH and AA depends on which condition predominates; following PNH treatment guidelines if hemolysis is the main symptom and AA treatment guidelines if bone marrow failure is severe (regardless of whether hemolysis is mild or moderate). The expert panel's recommendations on the monitoring and treatment of PNH in patients with AA are practical for healthcare systems in the Asia-Pacific region.
Subjects
Asia–Pacific
aplastic anemia
clone
flow cytometry
paroxysmal nocturnal hemoglobinuria
treatment
SDGs
Publisher
Multidisciplinary Digital Publishing Institute (MDPI)
Description
Article number 12160
Type
review