Diagnosis and therapy of hemophagocytic lymhohistiocytosis

Hemophagocytic lymphohistiocytosis

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome induced by excessive activation of lymphocytes and macrophages producing cytokine storm. The result might be the life threatening organ damage. HLH develops in patients with genetic abnormalities, hematologic malignancies, chronic inflammatory states, or infections. Most frequent clinical and laboratory features are fever, cytopenias, splenomegaly, hyperferritinemia and transaminitis. Early identification of a HLH trigger, such as malignancy or viral infection, is crucial for improving patient outcomes. Treatment has to be frequently started before the diagnosis of predisposing condition. Therapy of first choice might be in such situation the combination of corticosteroids and anakinra. Patients with primary HLH need the intensive therapy composed of corticosteroids and etoposide followed by allogeneic hematopoietic stem cell transplantation.