Hemophagocytic lymphohistiocytosis (HLH) is also known as hemophagocytic syndrome (HPS). It is mainly divided into primary HLH and secondary HLH. The former is mainly associated with related gene mutations, while the latter is mainly triggered by infection, tumor and autoimmune diseases. The main characteristic of HLH is that the immune dysfunction leads to reactive proliferation of monocytes/macrophages and the release of large amounts of cytokines, such as interferon-γ and tumor necrosis factor (TNF)-α under various pathogenic factors, leading to impaired and progressive aggravation of multi-organ and systems. The main clinical manifestations of HLH patients are fever, hepatosplenomegaly and pancytopenia, as well as serum ferritin elevation, hypertriglyceridemia, liver dysfunction, low fibrinogenemia and neurological symptoms etc.. HLH is a disease with complex clinical manifestation, rapid disease progression and high fatality rate. At present, the treatment methods are being explored, and better treatment methods can be found only by understanding the pathogenesis of HLH. To further understand the research progress of this disease, and to provide the basis for the improvement of treatment methods, this article summarizes the research status on the pathogenesis of HLH in recent years.
