The Challenging Aspect of Macrophage Activation Syndrome in the Setting of Sepsis or Systemic Inflammatory Response Syndrome (SIRS)

Overview

Journal Cureus

Specialty Biomedical Engineering

Date 2023 Apr 18

PMID 37069877

Authors

Said Benlamkaddem

Djoudline Doughmi

Imane Tlamcani

Mohamed Adnane Berdai

Mustapha Harandou

Affiliations

Soon will be listed here.

Abstract

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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