Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis, is a clinical condition characterized by an excessive inflammatory response in which the normal immune system is overstimulated by various factors that cause this defense mechanism to begin to impair normal organ function and engulf normal cells.
The early symptoms of hemophagocytic syndrome are fever, hepatomegaly and splenomegaly, complete blood cytopenia, and markedly elevated ferritin. The hemophagocytic syndrome is divided into two major categories, primary hemophagocytic syndrome and secondary hemophagocytic syndrome. Primary hemophagocytic syndrome is the result of a congenital genetic problem, where the mononuclear macrophage system and the hemophagocytes themselves are more activated than normal. There is a genetic correlation, and consanguineous marriage may lead to primary hemophagocytic syndrome in the next generation.
Secondary is generally seen in lymphoma, gastric cancer, lung cancer, and other neoplastic diseases that result in hemophagocytic activation. In addition, bacterial infections, viral and fungal infections, systemic lupus erythematosus, and related connective tissue diseases can also cause hemophagocytic activation, and an overreaction of hemophagocytes against bacteria can lead to damage to other organs. Hemophagocytic syndrome is a fatal disease with a very high mortality rate.