[New era of accurate diagnosis of hemophagocytic lymphohistiocytosis].

Hemophagocytic lymphohistiocytosis is a rapidly progressive and highly fatal disease with no specific clinical manifestations and multiple potential etiologies. Timely initiation of appropriate treatment is the key to improvement of the prognosis. Timely, accurate, and complete diagnosis of hemophagocytic lymphohistiocytosis requires the three-step principle, which is suspected diagnosis, confirmed diagnosis, and etiological diagnosis. With the development of new diagnostic technologies and methods, the establishment of a standardized diagnostic system and the construction of clinical pathways for hemophagocytic lymphohistiocytosis, the diagnostic efficiency of the fatal disease has been continuously improved, and hemophagocytic lymphohistiocytosis has entered an era of standardized, path-based and individualized accurate diagnosis.