Department of Hematology, Oncology and Internal Medicine, Medical University of Warsaw, Warsaw, Poland.
Department of Hematology, Oncology and Internal Medicine, Medical University of Warsaw, Warsaw, Poland
Pol Arch Intern Med. 2020 May 29;130(5):431-437. doi: 10.20452/pamw.15226. Epub 2020 Mar 5.
Hemophagocytic lymphohistiocytosis (HLH; also, hemophagocytic syndrome) occurs when an inflammatory reaction cannot stop on its own but continues to self‑ accelerate with positive feedback loops. If not interrupted, this pathomechanism leads to death. HLH in adults is usually diagnosed based on the HLH‑2004 criteria, but its confirmation should not stop the diagnostic process. Finding the triggering factor (especially malignancy) is of utmost importance. Treatment strongly depends on the established trigger and it is often based on the etoposide HLH‑94 protocol (adjusted for adults). Diagnostic workup should not unnecessarily delay the treatment since patients in severe or quickly deteriorating clinical condition require its fast initiation. Considering the progressive nature of HLH, time is extremely important. Prompt diagnosis and treatment, frequently made by an internal medicine specialist, is life‑saving. The aim of this review is to raise HLH awareness among internal medicine specialists and to provide advice on HLH management tailored for this group of physicians. Suggested approach is based on the latest recommendations by the Histiocyte Society and include novel insights from the authors' experience.
噬血细胞性淋巴组织细胞增生症（HLH；也称为噬血细胞综合征）是一种炎症反应无法自行停止，而是通过正反馈循环持续自我加速的疾病。如果不加以干预，这种病理机制会导致死亡。成人 HLH 通常根据 HLH-2004 标准进行诊断，但确诊不应停止诊断过程。寻找触发因素（尤其是恶性肿瘤）至关重要。治疗强烈依赖于已确定的触发因素，通常基于依托泊苷 HLH-94 方案（根据成人进行调整）。诊断性检查不应不必要地延迟治疗，因为严重或迅速恶化的临床状况的患者需要快速开始治疗。鉴于 HLH 的进行性性质，时间至关重要。由内科专家迅速做出的及时诊断和治疗是挽救生命的关键。本综述的目的是提高内科专家对 HLH 的认识，并为该组医生提供针对 HLH 管理的建议。建议的方法基于组织细胞协会的最新建议，并包含作者经验的新见解。
