Hemophagocytic lymphohistiocytosis

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a life threatening illness often associated with malignancy, rheumatologic and infectious diseases. HLH presents with fever and involvement of many organ systems with hepatosplenomegaly, lymphadenopathy, rash and neurologic manifestations. Anemia and thrombocytopenia along with elevated ferritin, abnormal liver enzymes level and deranged coagulation profile should point towards HLH. Though hemophagocytosis on bone marrow examination is not seen in all cases, infiltration of the bone marrow by activated macrophages is consistent with the diagnosis. Immunological investigations are not essential for initiation of therapy. Treatment aim is to interrupt the amplification cascades of cytokines and suppress the hyperinflammation.