Benlamkaddem Said, Doughmi Djoudline, Tlamçani Imane, Berdai Mohamed Adnane, Harandou Mustapha
Maternal and Pediatric Critical Care Unit, Hassan II University Hospital, Fez, MAR.
Faculty of Medicine and Pharmacy, Sidi Mohamed Ben Abdellah University, Fez, MAR.
Cureus. 2023 Mar 16;15(3):e36228. doi: 10.7759/cureus.36228. eCollection 2023 Mar.
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.
巨噬细胞活化综合征（MAS）是一种罕见但可能致命的疾病。其特征为高度炎症反应，包括与高细胞因子血症相关的免疫细胞（CD8 T细胞和NK细胞）增殖和活化。患者表现为发热、脾肿大和血细胞减少，骨髓中可见噬血细胞现象。它可进展为多器官功能衰竭综合征（MODS），类似脓毒症或全身炎症反应综合征（SIRS）。我们报告了一名8岁女孩的病例，她因家庭事故导致严重创伤入住儿科重症监护病房。尽管接受了适当治疗，但她在脓毒性休克的情况下仍持续发热。双血细胞减少、高铁蛋白血症、低纤维蛋白原血症和高甘油三酯血症提示为MAS，骨髓穿刺显示噬血细胞现象证实了这一诊断。随后在支持治疗和广谱抗生素治疗中加入了大剂量皮质激素治疗，结果良好。
