Macrolide-refractory Mycoplasma pneumoniae pneumonia and hemophagocytic lymphohistiocytosis: case report and literature review

Ma, Sung-Yun; Kim, Danbi; Lee, Juyoung; Cho, Kyoungsoon; Suh, Jin-Soon; Lee, Sang Yup

doi:10.22470/pemj.2020.00094

Cited by 2 publications

(1 citation statement)

References 16 publications

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“…In actual practice, a significant proportion of occult MAS (e.g., ~30% of sJIA flares and ~7% of refractory KD) progresses to overt MAS (Figure 2). In addition, occult forms of HLH or MAS may be more common than expected in infectious and malignant diseases [53,57].…”

Section: Occult Mas: Who Needs Screening?mentioning

confidence: 99%

Macrophage Activation Syndrome in Children: Update on Diagnosis and Treatment

Lee,

Bae,

Rhim

et al. 2024

Children

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Macrophage activation syndrome (MAS) is potentially fatal; so, early diagnosis and timely treatment are essential. However, detecting MAS is sometimes challenging because its principal features can be observed in other pediatric diseases that cause severe inflammation. Cytokine storm due to immune dysregulation represents the clinical and laboratory features of MAS that are included in the diagnostic criteria. Most cases of MAS occur as an underlying condition worsens and progresses. Therefore, a patient with autoimmune or autoinflammatory disease who shows unexplained clinical deterioration despite appropriate management should be considered at high risk for MAS (i.e., occult MAS). The basic principles of treatment are control of triggering factors, supportive care, and relief of hyperinflammation. Systemic steroids and cyclosporine A are frequently used as a first-line treatment. For the treatment of refractory MAS, cytokine-specific biologic agents such as anakinra have recently become preferred over traditional immunosuppressive agents such as etoposide. MAS might be underrecognized in pediatric patients with infectious and inflammatory diseases due to its diverse clinical presentations. Clinical suspicion of MAS is of the utmost importance for early recognition of the disease.

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Section: Occult Mas: Who Needs Screening?mentioning

confidence: 99%

Macrophage Activation Syndrome in Children: Update on Diagnosis and Treatment

Lee,

Bae,

Rhim

et al. 2024

Children

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Kawasaki Disease Shock Syndrome and Macrophage Activation Syndrome: A Case Report

Kim,

Cho

et al. 2023

Kawasaki Dis

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As a major organ dysfunction, some patients with Kawasaki disease (KD) present with hypotension or shock, known as KD shock syndrome (KDSS). Organ dysfunction is also seen in macrophage activation syndrome (MAS), complicating KD (MAS-KD). Both KDSS and MAS are rare but potentially life-threatening complications of KD that require early recognition. In practice, however, KDSS and MAS can sometimes be overlooked in patients with KD. Here, we describe a 13-year-old boy who presented with prolonged fever, rash, and red eyes, and subsequently developed hypotension. He was diagnosed with KDSS and received inotropic agents and intravenous immunoglobulin (IVIG) treatment. However, his clinical and laboratory features deteriorated, and he met the diagnostic criteria for MAS. KDSS and MAS-KD share many overlapping features in terms of organ dysfunction. A diagnosis of KDSS is made when a patient with KD shows cardiac dysfunction, such as shock. If a KD patient displays multi-organ dysfunction, such as hematologic, hepatic, renal, or neurologic symptoms, they should be diagnosed with MAS-KD. As demonstrated in this case report, some KD patients may present with both cardiac and multi-organ dysfunction and require prompt and proper treatment for both.

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Macrolide-refractory Mycoplasma pneumoniae pneumonia and hemophagocytic lymphohistiocytosis: case report and literature review

Cited by 2 publications

References 16 publications

Macrophage Activation Syndrome in Children: Update on Diagnosis and Treatment

Macrophage Activation Syndrome in Children: Update on Diagnosis and Treatment

Kawasaki Disease Shock Syndrome and Macrophage Activation Syndrome: A Case Report

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