Macrophage activation syndrome: A cause of unresponsive malaria

Abstract

Macrophage activation syndrome (MAS) results from inappropriate proliferation and activation of cells of the macrophage-histiocytic lineage. Severe infections, autoimmune diseases and malignancies are known to trigger MAS in otherwise healthy individuals. We describe a case of complicated mixed malaria (Plasmodium falciparum and Plasmodium vivax) with MAS in a 5-year-old immunocompetent girl who presented with fever, severe anemia, jaundice, and hepatosplenomegaly. Multiple packed red cells transfusions were required for her repeatedly falling hemoglobin. Fever was persistent despite antimalarials without any coexistent bacterial or viral infection. Laboratory findings included variable cytopenia, hyperbilirubinemia, hyperferritinemia, hypertriglyceridemia, and megaloblatic picture on bone marrow. A final diagnosis of macrophage activation syndrome with megaloblastic anemia in a child with severe mixed malaria was made. There was a dramatic response to steroid treatment with improvement in clinical condition. This report endorses the use of steroids in malaria associated MAS when there is no clinical improvement with antimalarials alone.

Keywords

• Macrophage activation
• malaria
• megaloblastic anemia