Department of Clinical Chemistry. LHUB-ULB, Université Libre de Bruxelles (ULB), 322, Rue Haute, 1000, Brussels, Belgium.
Department of Haematology. LHUB-ULB, Université Libre de Bruxelles (ULB), 322, Rue Haute, 1000, Brussels, Belgium.
Ann Hematol. 2022 Jul;101(7):1485-1491. doi: 10.1007/s00277-022-04845-4. Epub 2022 Apr 22.
Hereditary spherocytosis (HS) is the most common inherited chronic haemolytic anaemia in Northern Europe. During the last decade, additional erythrocyte and reticulocyte parameters have been developed on last-generation haematology analysers, leading to many publications about their effectiveness as a HS screening tool. For the first time on an independent cohort, we evaluated and compared the effectiveness of six published algorithms for the screening of HS using the UniCel DxH800 (Beckman-Coulter) and the XN-9000 (Sysmex) and determined which algorithm could be the most suitable in our daily clinical practice. A total of 95 EDTA samples were analysed prospectively on both haematology analysers. These included 11 confirmed HS patients and 84 non-HS patients. The specific reticulocyte parameters used on the DxH800 were mean reticulocyte volume, immature reticulocyte fraction and mean sphered cell volume, and on the XN-9000 were hypohaemoglobinised erythrocytes, microcytic erythrocytes and immature reticulocyte fraction. The three algorithms using parameters specific to Beckman-Coulter analysers provided a sensitivity of 100% with various specificities, ranging from 7.1 to 73.8%. The three algorithms published based on the parameters specific to Sysmex showed much lower performances, i.e. out of the 11 patients with HS, between one to five patients were screened as negative for HS. However, 100% sensitivity and specificity were reached using the EMA binding test concomitantly with those three algorithms. The algorithms using reticulocyte and erythrocyte parameters offered by the recent analysers are promising options as a HS first-tier screening tool. Nevertheless, they must be evaluated by each laboratory on their own analyser before implementation.
遗传性球形红细胞增多症（HS）是北欧最常见的遗传性慢性溶血性贫血。在过去十年中，上一代血液学分析仪开发了额外的红细胞和网织红细胞参数，这导致了许多关于其作为 HS 筛查工具的有效性的出版物。我们首次在独立队列中评估和比较了使用 Beckman-Coulter 的 UniCel DxH800 和 Sysmex 的 XN-9000 对 6 种已发表的 HS 筛查算法的有效性，并确定了哪种算法最适合我们的日常临床实践。总共对这两台血液学分析仪进行了 95 份 EDTA 样本的前瞻性分析。这些样本包括 11 名确诊的 HS 患者和 84 名非 HS 患者。DxH800 上使用的特定网织红细胞参数是平均网织红细胞体积、未成熟网织红细胞分数和平均球形红细胞体积，而 XN-9000 上使用的是低血红蛋白红细胞、小细胞红细胞和未成熟网织红细胞分数。使用 Beckman-Coulter 分析仪特定参数的三个算法提供了 100%的敏感性，特异性范围为 7.1%至 73.8%。基于 Sysmex 特定参数的三个算法的性能要低得多，即 11 名 HS 患者中，有 1 至 5 名患者被筛查为 HS 阴性。然而，同时使用这三个算法和 EMA 结合试验可达到 100%的敏感性和特异性。最近的分析仪提供的网织红细胞和红细胞参数算法是作为 HS 一线筛查工具的有前途的选择。然而，在实施之前，每个实验室都必须在自己的分析仪上进行评估。
