Department of Pediatrics, Inje University Haeundae Paik Hospital, Busan, Korea.
Department of Pediatrics, Keimyung University Dongsan Hospital, Keimyung University School of Medicine, Daegu, Korea.
J Korean Med Sci. 2024 May 13;39(18):e162. doi: 10.3346/jkms.2024.39.e162.
Hereditary hemolytic anemia (HHA) is considered a group of rare hematological diseases in Korea, primarily because of its unique ethnic characteristics and diagnostic challenges. Recently, the prevalence of HHA has increased in Korea, reflecting the increasing number of international marriages and increased awareness of the disease. In particular, the diagnosis of red blood cell (RBC) enzymopathy experienced a resurgence, given the advances in diagnostic techniques. In 2007, the RBC Disorder Working Party of the Korean Society of Hematology developed the Korean Standard Operating Procedure for the Diagnosis of Hereditary Hemolytic Anemia, which has been continuously updated since then. The latest Korean clinical practice guidelines for diagnosing HHA recommends performing next-generation sequencing as a preliminary step before analyzing RBC membrane proteins and enzymes. Recent breakthroughs in molecular genetic testing methods, particularly next-generation sequencing, are proving critical in identifying and providing insight into cases of HHA with previously unknown diagnoses. These innovative molecular genetic testing methods have now become important tools for the management and care planning of patients with HHA. This review aims to provide a comprehensive overview of recent advances in molecular genetic testing for the diagnosis of HHA, with particular emphasis on the Korean context.
遗传性溶血性贫血 (HHA) 在韩国被认为是一组罕见的血液系统疾病,主要是由于其独特的民族特征和诊断挑战。最近,韩国 HHA 的患病率有所增加,反映出国际婚姻的增加和对该病的认识提高。特别是,随着诊断技术的进步,红细胞 (RBC) 酶病的诊断重新受到关注。2007 年,韩国血液学会 RBC 疾病工作组制定了《遗传性溶血性贫血诊断韩国标准操作规程》,此后一直在不断更新。最近的韩国 HHA 诊断临床实践指南建议在分析 RBC 膜蛋白和酶之前,先进行下一代测序作为初步步骤。分子遗传学检测方法的最新突破,尤其是下一代测序,对于识别和深入了解以前诊断不明的 HHA 病例至关重要。这些创新的分子遗传学检测方法现已成为 HHA 患者管理和护理计划的重要工具。本综述旨在全面介绍用于诊断 HHA 的分子遗传学检测的最新进展,特别强调韩国的情况。