Monitoring and Treatment of Paroxysmal Nocturnal Hemoglobinuria in Patients with Aplastic Anemia in Asia: An Expert Consensus

Raymond Siu Ming Wong, Jun Ho Jang, Lily Lee Lee Wong, Jin Seok Kim, Ponlapat Rojnuckarin, Yeow Tee Goh, Yasutaka Ueda, Wen Chien Chou, Jong Wook Lee, Yuzuru Kanakura, Tzeon Jye Chiou

Research output: Contribution to journalReview articlepeer-review

1 Citation (Scopus)

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.

Original languageEnglish
Article number12160
JournalInternational journal of molecular sciences
Volume25
Issue number22
DOIs
Publication statusPublished - Nov 2024

Keywords

  • aplastic anemia
  • Asia–Pacific
  • clone
  • flow cytometry
  • paroxysmal nocturnal hemoglobinuria
  • treatment

ASJC Scopus subject areas

  • Catalysis
  • Molecular Biology
  • Spectroscopy
  • Computer Science Applications
  • Physical and Theoretical Chemistry
  • Organic Chemistry
  • Inorganic Chemistry

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