Haemophagocytic syndrome associated with infections

Haemophagocytic syndromes (HS) are the clinical manifestation of an increased macrophagic activity with haemophagocytosis. Pathophysiology is related to a deregulation of T-lymphocytes and excessive production of cytokines. The main clinicobiological features are fever, hepatosplenomegaly, adenopathies, skin rash, neurological features, cytopenias, hypertriglyceridaemia, hyperferritinaemia and coagulopathy. Diagnosis is based on examination of the bone marrow which shows benign histiocytes actively phagocytosing haemopoietic cells. Acquired HS are mostly associated with an underlying disease such as immunodeficiency, haematological neoplasias and autoimmune diseases. Infection-associated HS was originally described by Risdall in 1979, in viral disease. Since the initial description HS has also been documented in patients with bacterial, parasitic or fungal infections. Epstein-Barr virus (EBV) is the causative agent in most cases. In EBV-associated HS, which sometimes has a fatal course, unregulated T-cell reaction or uncontrolled B-cell proliferation may release cytokines. Management of HS consists of early diagnosis, careful screening for, and prompt treatment of, infections and detection and therapy of any underlying disease. Prognosis of infection-associated haemophagocytic syndrome (IAHS) is better than that in other types of secondary HS. Management of cytokine imbalance should be useful to improve the outcome and reduce the mortality rate in these cases.