Macrophage activation syndrome in a child with varicella: a case report

Macrophage activation syndrome is a severe, potentially fatal condition that belongs to the group of hemophagocytic lymphohistiocytosis. It occurs when the immune system becomes overactive and begins to “work at maximum capacity” in an uncontrolled manner. It can occur in autoimmune conditions as well as infectious diseases. It is characterized by persistent febrile fever, lymphadenopathy, hepatosplenomegaly with signs of liver failure, hemorrhagic syndrome, pancytopenia, and hyperferritinemia. Timely diagnosis and urgent treatment initiation are crucial for successfully managing patients with macrophage activation syndrome. The clinical case demonstrates the complexity of macrophage activation syndrome diagnosis. On the background of the infectious process caused by the Varicella zoster virus, hyperactivation of the immune system occurred with the development of hyperinflammatory syndrome, and hypercytokinemia, which required the appointment of emergency therapy.

A case of macrophage activation syndrome in a 10-year-old Ukrainian girl is described. After Varicella manifestation the child’s general condition worsened, she developed oliguria, confluent hemorrhagic rashes around the ankle–foot joints, and signs of epidermal desquamation on the lateral surface of the knee and hip joints. Increasing thrombocytopenia, anemia, hypofibrinogenemia, and hyperferritinemia were detected. After prescribing antibiotic therapy, glucocorticosteroids, and intravenous human immunoglobulin, the child’s condition did not improve and oliguria persisted; therefore, the child was transferred to hemodialysis, after which kidney function was restored. In the place of hemorrhages on the lower legs, surface necrosis developed, which regressed against the background of treatment, leaving pigmentation and scars.

The case of macrophage activation syndrome in a child during Varicella convalescence is presented. Extensive hemorrhagic rashes on the skin of the lower extremities with the development of superficial necrosis was the main clinical peculiarity, which led to misdiagnosis with meningococcal infection. Macrophage activation syndrome was diagnosed owing to the presence of liver involvement with cytolysis syndrome, kidney damage with acute renal failure, thrombocytopenia, anemia, hypofibrinogenemia, pancytopenia, and a high level of ferritin.