How to Treat Cytomegalovirus-induced Hemophagocytic Lymphohistiocytosis in a Child with Leukemia

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by a hyperinflammatory state due to an aberrant activation of the immune cells. It can be familial or secondary to malignancy, autoimmune or metabolic diseases. Most HLH cases are triggered by infection. Histiocyte society suggested HLH-2004 protocol for diagnosis and treatment of both forms. Here, we present a three-year-old girl with B-cell acute lymphoblastic leukemia who developed HLH secondary to cytomegalovirus infection during maintenance therapy. She was successfully treated without needing full HLH protocol therapy. We discuss modified therapy for this specific group of HLH, summarizing 5 other similar cases in the literature.