期刊
CUREUS JOURNAL OF MEDICAL SCIENCE
卷 15, 期 3, 页码 -出版社
SPRINGERNATURE
DOI: 10.7759/cureus.36228
关键词
thrombocytopenia; bone marrow puncture; hemophagocytosis; sirs; sepsis; macrophage activation syndrome
Macrophage activation syndrome (MAS) is a rare but potentially fatal disease characterized by hyperinflammation and immune cell activation. It can progress to multiorgan failure, mimicking sepsis. We report the case of an 8-year-old girl with MAS following a major trauma, who responded well to corticosteroid therapy and broad-spectrum antibiotics.
Macrophage activation syndrome (MAS) is a rare but potentially fatal disease. It is characterized by hyperinflammation, including the proliferation and activation of immune cells (CD8 T cells and NK cells) associated with hypercytokinemia. Patients present with fever, splenomegaly, and cytopenia, associated with a hemophagocytosis picture in the bone marrow. It can progress to a multiorgan failure syndrome (MODS), mimicking sepsis or a systemic inflammatory response syndrome (SIRS). We report the case of an 8-year-old girl admitted to the pediatric intensive care unit for the management of major trauma due to a domestic accident. She presented with a protracted fever in the context of a septic shock, despite appropriate treatment. The association with bicytopenia, hyperferritinemia, hypofibrinogenemia, and hypertriglyceridemia was suggestive of MAS which was confirmed by a bone marrow puncture showing hemophagocytosis. A Bolus of corticotherapy was then added to the supportive treatment and broad-spectrum antibiotherapy, with a good outcome.
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