Bseiso Omair, Zahdeh Anas, Isayed Obay, Mahagna Seewar, Bseiso Anan
College of Medicine, Hebron University, Hebron, PSE.
College of Medicine, An-Najah National University, Nablus, PSE.
Cureus. 2022 Dec 31;14(12):e33175. doi: 10.7759/cureus.33175. eCollection 2022 Dec.
This review article describes the pathophysiology of hemophagocytic lymphohistiocytosis (HLH). The condition is characterized by excessive stimulation of inflammatory cytokines, lymphocytes, and macrophages, leading to hyperinflammatory disorder with immune dysfunction. The main clinical and diagnostic features include fever ≥38.5°C, splenomegaly, hyperferritinemia, cytopenia, hypofibrinogenemia, hemophagocytosis on the bone marrow, low or absent of natural killer (NK) cell activity, and elevated soluble CD25. Various immunological and inflammatory mechanisms are involved in the pathogenesis of HLH. Moreover, the condition can result in multisystem organ failure, contributing to the high mortality rate in hospital settings. A thorough literature search was conducted by collecting data from multiple articles published on PubMed, Medline, and Google Scholar. The article discusses the cellular and molecular pathways that lead to HLH. Due to the high rate of morbidity and mortality, early diagnosis needs to be established. More research pertaining to molecular biology, immunology, and the genetics of HLH is needed to explore the effective management and treatment of this rare disorder.
这篇综述文章描述了噬血细胞性淋巴组织细胞增生症（HLH）的病理生理学。该病症的特征是炎性细胞因子、淋巴细胞和巨噬细胞受到过度刺激，导致伴有免疫功能障碍的高炎症性疾病。主要的临床和诊断特征包括体温≥38.5°C、脾肿大、高铁蛋白血症、血细胞减少、低纤维蛋白原血症、骨髓噬血细胞现象、自然杀伤（NK）细胞活性降低或缺失以及可溶性CD25升高。HLH的发病机制涉及多种免疫和炎症机制。此外，该病症可导致多系统器官衰竭，这也是导致医院环境中高死亡率的原因。通过收集在PubMed、Medline和谷歌学术上发表的多篇文章的数据进行了全面的文献检索。本文讨论了导致HLH的细胞和分子途径。由于发病率和死亡率较高，需要尽早确诊。需要开展更多关于HLH分子生物学、免疫学和遗传学的研究，以探索对这种罕见病症的有效管理和治疗方法。
