Lymphohistiocyosis

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a life threatening condition characterized by uncontrolled hyperinflammation caused by various inherited or acquired immunodeficiency states. Cardinal symptoms are prolonged fever, hepatosplenomegaly, lymphadenopathy, rash, and central nervous system symptoms. Laboratory findings include high triglycerides, ferritin, transaminases, bilirubin and alpha chain of the soluble IL2 receptor, and decreased fibrinogen. A hallmark of HLH is impaired or absent function of natural killer cells and cytotoxic T cells. Hemophagocytosis, which has given the disease its name, is found in only a minority of cases at presentation, and its occurrence increases as the disease progresses.

Keywords

• Etoposide
• hemophagocytic lymphohistiocytosis
• NK cells