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Case Reports
. 2023 Mar 16;15(3):e36228.
doi: 10.7759/cureus.36228. eCollection 2023 Mar.

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

Said Benlamkaddem  1   2 , Djoudline Doughmi  1   2 , Imane Tlamçani  3   2 , Mohamed Adnane Berdai  1   2 , Mustapha Harandou  1   2
Affiliations
Case Reports

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

Said Benlamkaddem et al. Cureus. .

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.

Keywords: bone marrow puncture; hemophagocytosis; macrophage activation syndrome; sepsis; sirs; thrombocytopenia.

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Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. Hemophagocytic cells in bone marrow smear (MGG; Optical microscope x100).
Macrophages phagocytosing mature red blood cells (RBC) and nucleated RBC precursors, neutrophils and granulocyte precursors, plasma cells, and lymphocytes (A-B). Macrophage, including a red blood cell and platelets (C). Example of a histocyte-ingested platelet that we do not consider to be a definite hemophagocyte (D). MGG: May-Grünwald Giemsa coloration
See this image and copyright information in PMC

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