Otrock Zaher K, Hock Karl G, Riley Sarah B, de Witte Theo, Eby Charles S, Scott Mitchell G
Department of Pathology and Immunology, Washington University School of Medicine, 660 S. Euclid Ave, Campus Box 8118, St. Louis, MO, 63110, USA.
Forensic Toxicology Laboratory, Saint Louis University, St Louis, MO, USA.
Ann Hematol. 2017 Oct;96(10):1667-1672. doi: 10.1007/s00277-017-3072-0. Epub 2017 Jul 31.
Hemophagocytic lymphohistiocytosis (HLH) is a rare, potentially fatal, syndrome of excessive and ineffective activation of the immune system. The majority of the reported data on HLH is from pediatric patients and lacks specificity. This makes HLH diagnosis challenging especially in adults where HLH is triggered by many conditions and can resemble many disease entities. Elevated ferritin is one of the diagnostic criteria for HLH. We determined the conditions associated with elevated ferritin at our medical center to assess how specific ferritin is for predicting HLH. We retrospectively reviewed all ferritin results >10,000 μg/L in pediatric and adult patients. The most common condition associated with elevated ferritin was hematologic malignancy in adults (25.7%) and HLH in pediatric patients (48.9%). HLH was diagnosed in 14.2% of adults and 48.9% of children with ferritin >10,000 µg/L. Hyperferritinemia occurs in a variety of conditions and is not specific for adult or pediatric HLH. Common causes of elevated ferritin should be considered before entertaining the possibility of HLH, especially in adult patients.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见的、潜在致命的免疫系统过度激活且无效的综合征。关于HLH的大多数报告数据来自儿科患者，缺乏特异性。这使得HLH的诊断具有挑战性，尤其是在成人中，HLH由多种情况引发，且可能类似于许多疾病实体。铁蛋白升高是HLH的诊断标准之一。我们在我们的医疗中心确定了与铁蛋白升高相关的情况，以评估铁蛋白对预测HLH的特异性如何。我们回顾性地审查了儿科和成人患者中所有铁蛋白结果>10,000μg/L的情况。与铁蛋白升高相关的最常见情况在成人中是血液系统恶性肿瘤（25.7%），在儿科患者中是HLH（48.9%）。在铁蛋白>10,000µg/L的成人患者中，14.2%被诊断为HLH，在儿童患者中这一比例为48.9%。高铁蛋白血症在多种情况下都会出现，并非成人或儿科HLH所特有。在考虑HLH的可能性之前，尤其是在成人患者中，应考虑铁蛋白升高的常见原因。
