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Whole-cell MALDI-TOF Mass Spectrometry is an Accurate and Rapid Method to Analyze Different Modes of Macrophage Activation
Published on: December 26, 2013
Macrophage Activation Syndrome
P Babu Raj1, B L Harikrishnan2, Roshan Mampilly3
1Professor and Unit Chief.
Abstract:
Macrophage Activation Syndrome (MAS) occurs as a severe life-threatening complication of several chronic rheumatic diseases. It is more frequent with systemic onset juvenile arthritis and adult onset Still's disease.1 It can be primary, infection related, malignancy associated or autoimmune3. We report a case of Macrophage Activation Syndrome presenting as pyrexia of unknown origin (PUO) and pancytopenia in the absence of any known triggering factor.
Insights
Macrophage Activation Syndrome (MAS), a severe complication of rheumatic diseases, can present unusually. This case highlights MAS manifesting as fever and low blood counts without a clear cause.
Area of Science:
- Rheumatology
- Hematology
- Immunology
Background:
- Macrophage Activation Syndrome (MAS) is a life-threatening complication associated with chronic rheumatic diseases.
- It is particularly prevalent in systemic onset juvenile arthritis and adult onset Still's disease.
- MAS can be triggered by various factors including infections, malignancies, and autoimmune conditions.
Observation:
- This report details a case where MAS presented as pyrexia of unknown origin (PUO).
- The patient also exhibited pancytopenia, a significant reduction in all blood cell types.
- Crucially, no known triggering factor for MAS was identified in this instance.
Findings:
- The study identifies an atypical presentation of Macrophage Activation Syndrome.
- It emphasizes the diagnostic challenge posed by MAS when common triggers are absent.
- The case underscores the importance of considering MAS in unexplained fever and pancytopenia.
Implications:
- This case broadens the understanding of MAS presentations.
- It suggests that MAS should be considered even in the absence of typical triggers.
- Further research may elucidate rarer etiologies and diagnostic pathways for MAS.

