Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA;
Department of Biostatistics and Computational Biology, Dana-Farber Cancer Institute, Boston, MA; and.
Blood. 2015 Mar 5;125(10):1548-52. doi: 10.1182/blood-2014-10-602607. Epub 2015 Jan 8.
Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome of uncontrolled immune activation that has gained increasing attention during the last decade. The diagnosis of HLH is based on a constellation of clinical and laboratory abnormalities, including elevated serum ferritin levels. In the pediatric population, marked hyperferritinemia is specific for HLH. To determine what conditions are associated with profoundly elevated ferritin in the adult population, we performed a retrospective analysis in a large academic health care system. We identified 113 patients with serum ferritin levels higher than 50,000 µg/L. The most frequently observed conditions included renal failure, hepatocellular injury, infections, and hematologic malignancies. Our results suggest that marked hyperferritinemia can be seen in a variety of conditions and is not specific for HLH in adults.
噬血细胞性淋巴组织细胞增生症(HLH)是一种罕见的失控性免疫激活综合征,在过去十年中受到越来越多的关注。HLH 的诊断基于一系列临床和实验室异常,包括血清铁蛋白水平升高。在儿科人群中,明显的高血铁蛋白症是 HLH 的特异性表现。为了确定在成年人群中哪些疾病与铁蛋白显著升高相关,我们在一个大型学术医疗系统中进行了回顾性分析。我们确定了 113 例血清铁蛋白水平高于 50,000µg/L 的患者。最常观察到的疾病包括肾衰竭、肝细胞损伤、感染和血液系统恶性肿瘤。我们的结果表明,明显的高血铁蛋白症可见于多种疾病,在成人中并不特异性提示 HLH。