Department of Pathology, University of California San Francisco, San Francisco, CA, USA.
Department of Pathology, Stanford University, Stanford, CA, USA.
Histopathology. 2022 Mar;80(4):616-626. doi: 10.1111/his.14591. Epub 2022 Feb 10.
Haemophagocytic lymphohistiocytosis (HLH) is a complex, often under-recognised hyperinflammatory immune dysregulation syndrome arising in a diverse range of clinical scenarios and conditions. The accurate and timely diagnosis of HLH is crucial for patient survival, and usually requires a high level of clinical suspicion. The histological corollary to clinical HLH-haemophagocytosis-is neither necessary nor sufficient for the diagnosis of HLH, as it may be seen in a variety of reactive conditions and may be absent in true HLH. Nevertheless, the finding of haemophagocytosis in specific clinical situations should prompt consideration of HLH and further testing to exclude the condition. Although haemophagocytosis is traditionally described in bone marrow, identification of it in other tissues, including lymphoid, splenic, liver or neural tissue, can contribute importantly to the overall recognition of HLH. In this review we discuss the underlying pathophysiology and aetiologies of HLH, and the morphological aspects of haemophagocytosis and its associated histological findings in different tissues, and give a brief overview of diagnostic criteria and clinical evaluation.
噬血细胞性淋巴组织细胞增生症（HLH）是一种复杂的、常被低估的、过度活跃的免疫失调综合征，可发生于多种临床情况和疾病中。准确、及时地诊断 HLH 对患者的生存至关重要，通常需要高度的临床怀疑。HLH 的组织学对应物——噬血细胞现象——对于 HLH 的诊断既不是必需的，也不是充分的，因为它可能在多种反应性疾病中出现，也可能在真正的 HLH 中缺失。然而，在特定临床情况下发现噬血细胞现象应促使考虑 HLH，并进一步检查以排除该疾病。尽管噬血细胞现象传统上在骨髓中描述，但在其他组织中（包括淋巴组织、脾脏、肝脏或神经组织）识别噬血细胞现象对全面认识 HLH 具有重要意义。在这篇综述中，我们讨论了 HLH 的潜在病理生理学和病因，以及噬血细胞现象及其在不同组织中的相关组织学发现的形态学方面，并简要概述了诊断标准和临床评估。
