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Investigation of Macrophage Polarization Using Bone Marrow Derived Macrophages
Published on: June 23, 2013
Macrophage activation syndrome
1Pediatria II, Istituto G. Gaslini, Università di Genova, Italy. angeloravelli@ospedale-gaslini.ge.it
Abstract:
Macrophage activation syndrome (MAS) is a serious complication of childhood systemic inflammatory disorders that is thought to be caused by excessive activation and proliferation of T lymphocytes and macrophages. Recent findings in hemophagocytic lymphohistiocytosis, a disease that is clinically similar to MAS, highlight the possible pathogenetic role of a defective function of perforin, a protein involved in the cytolytic processes and control of lymphocyte proliferation. Although the clinical features of MAS have been well documented, early diagnosis can be difficult. Measurement of the serum ferritin level may assist in the diagnosis and may be a useful indicator of disease activity, therapy response, and prognosis. The recognition that MAS belongs to the secondary or reactive hemophagocytic syndromes has led to the proposal to rename it according to the contemporary classification of histiocytic disorders. Cyclosporin A has been found effective in patients with corticosteroid-resistant MAS. A recent report has suggested that etanercept may be a useful adjunctive therapeutic agent.
Insights
Macrophage activation syndrome (MAS) is a severe inflammatory disorder in children. Early diagnosis is challenging, but serum ferritin levels may aid in identifying MAS and monitoring treatment effectiveness.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Hematology
Background:
- Macrophage activation syndrome (MAS) is a life-threatening complication of childhood systemic inflammatory disorders.
- It involves excessive T lymphocyte and macrophage activation, potentially linked to defective perforin function, similar to hemophagocytic lymphohistiocytosis.
Purpose of the Study:
- To review the diagnostic challenges and therapeutic strategies for Macrophage Activation Syndrome (MAS).
- To highlight the role of serum ferritin in MAS diagnosis and management.
Main Methods:
- Literature review of Macrophage Activation Syndrome (MAS) and related conditions.
- Analysis of diagnostic markers and therapeutic interventions, including novel agents.
Main Results:
- Early diagnosis of MAS remains difficult despite well-documented clinical features.
- Serum ferritin levels are a valuable indicator for MAS diagnosis, disease activity, treatment response, and prognosis.
- Cyclosporin A shows efficacy in corticosteroid-resistant MAS.
- Etanercept is suggested as a potential adjunctive therapy.
Conclusions:
- MAS is increasingly recognized as a secondary hemophagocytic syndrome, prompting reclassification.
- Serum ferritin measurement is crucial for effective MAS management.
- Current and emerging therapies offer hope for managing this severe pediatric condition.
