Division of Transfusion Medicine and Hemostasis, Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
Children's Health System, Dallas, Texas, USA.
Transfusion. 2024 Oct;64(10):1822-1829. doi: 10.1111/trf.17995. Epub 2024 Oct 3.
Hyperhemolysis syndrome (HHS) is a catastrophic anemia characterized by destruction of both donor and patient red blood cells (RBC). HHS occurs after transfusion and can cause significant morbidity and mortality. Given the difficulty in diagnosing and managing this process, we provide a detailed overview of our treatment protocol.
Members of the Transfusion Medicine and Hematology faculty at our institution collaborated in an iterative process to produce a consensus approach to patients with HHS.
We present diagnostic criteria for HHS: recent transfusion within past 7 days (up to 21 days), rapid hemoglobin decline to below the pretransfusion level (usually hemoglobin drop >25% from pretransfusion), a significant decrease in HbA% (in patients with sickle cell disease or beta thalassemia), low or decreasing reticulocyte count in a patient with worsening anemia, and laboratory evidence of hemolysis. We also describe an in-depth approach to management focusing on optimizing hematopoiesis while dampening the immune response.
We provide a comprehensive approach to the diagnosis and management of HHS based on contemporary literature and clinical experience designed to optimize outcomes for patients.
高溶血性输血反应综合征（HHS）是一种以供者和患者的红细胞（RBC）均被破坏为特征的灾难性贫血。HHS 发生在输血后，可导致严重的发病率和死亡率。鉴于诊断和处理这一过程的难度，我们提供了详细的治疗方案概述。
我们机构的输血医学和血液学教员成员在一个迭代过程中合作，制定了针对 HHS 患者的共识方法。
我们提出了 HHS 的诊断标准：最近在过去 7 天内（最多 21 天）进行了输血，血红蛋白迅速下降至低于输血前水平（通常血红蛋白下降超过输血前的 25%），HbA%显著下降（镰状细胞病或β地中海贫血患者），贫血恶化时网织红细胞计数低或减少，以及溶血的实验室证据。我们还描述了一种深入的管理方法，重点是优化造血功能，同时抑制免疫反应。
我们根据当代文献和临床经验提供了一种全面的 HHS 诊断和管理方法，旨在优化患者的治疗结果。
