Sir Y.K. Pao Centre for Cancer & Department of Medicine and Therapeutics, Prince of Wales Hospital, The Chinese University of Hong Kong, 30-32 Ngan Shing Street, Sha Tin, Hong Kong SAR, China.
Division of Hematology-Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, 2066 Seobu-Ro, Suwon 16419, Republic of Korea.
Int J Mol Sci. 2024 Nov 13;25(22):12160. doi: 10.3390/ijms252212160.
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.
阵发性睡眠性血红蛋白尿症（PNH）克隆可在相当一部分再生障碍性贫血（AA）患者中被发现。国家和国际指南均建议在 AA 诊断时筛查 PNH 克隆。在本报告中，一组医学专家讨论了当前的最佳实践，并为亚太地区 AA 患者的 PNH 管理提供了建议。每次血液检查都应测量血浆/血清乳酸脱氢酶（LDH）水平和网织红细胞计数。应通过流式细胞术定期监测 PNH 克隆大小，在 LDH 水平升高±网织红细胞计数或出现血栓形成等症状时按需进行检测。监测 PNH 克隆可以指导 AA 初始治疗的选择，尽管流式细胞术具有资源影响，这可能在一些亚太国家构成挑战。PNH 和 AA 患者的治疗取决于哪种情况占主导地位；如果溶血是主要症状，则遵循 PNH 治疗指南，如果骨髓衰竭严重（无论溶血是轻度还是中度），则遵循 AA 治疗指南。专家组关于 AA 患者 PNH 监测和治疗的建议适用于亚太地区的医疗保健系统。
