Unraveling the Diagnostic and Therapeutic Dilemma in Macrophage Activation Syndrome: A Case Series

Aim and background: Macrophage activation syndrome (MAS) is a hyper-inflammatory condition with a severe cytokine storm which is not an uncommon complication of various diseases in adult patients, mostly presenting in acute medical units.Here, we present a case series of four patients taken from the acute medical units of different tertiary care hospitals in Eastern India.Case presentations: The most common underlying conditions of MAS are sepsis, malignancies, and various rheumatological disorders.It has varied presentations and frequently overlaps with various other diseases, and considering the clinical inertia from both the clinicians and patients, pose a great difficulty in proper diagnosis, leading to high rate of mortality in such patients.A high degree of clinical suspicion is the key to early diagnosis and management.Conclusion: A strong degree of clinical suspicion is very important for early diagnosis and reduced mortality.The management plan too depends on individualistic approach and should be as per a joint consensus whether restricting to only aggressively addressing the underlying causes or addition of immunosuppressive in the form of monotherapy with steroid or by a combination of steroid and other immunosuppressants.Clinical significance: Understanding the clinical presentation of MAS is primarily based on observations in systemic juvenile idiopathic arthritis and pediatric hemophagocytic lymphohistiocytosis (HLH).Classical features include fever, hepatosplenomegaly, cytopenia, high ferritin, raised triglycerides, and hemophagocytosis in the bone marrow, spleen, or lymph nodes.However, it quite often presents with atypical and overlapping features.In addition to it mere trivial subclinical to severe and potentially life-threatening episodes can be the presenting features of MAS.