Hemophagocytic syndrome

The common basis for hemophagocytic syndromes are genetic or acquired immune defects which lead to an exaggerated but ineffective immune response with high levels of inflammatory cytokines. Cardinal symptoms are prolonged, antibiotic resistant fever, hepatosplenomegaly and bicytopenia or pancytopenia. Hemophagocytosis, which gave hemophagocytic syndrome its name, often develops later during the course of the disease. Characteristic laboratory values are high concentrations of ferritin, triglycerides, lactate dehydrogenase, bilirubin, and transaminases, and low levels of fibrinogen, or a generalized coagulation disorder. In contrast to a normal infection, the symptoms are more pronounced and, most important, they are progressive. Timely treatment with immunomodulatory and cytostatic drugs can suppress hyperinflammation and thus be lifesaving.