Macrophage activation syndrome: experience from a tertiary referral centre.

Over a period of 3 years, 13 patients with Macrophage Activation Syndrome were seen. Most had underlying connective tissue disease or malignancy. High-grade fever, cytopenia and elevated transaminases were the common presenting manifestations. Elevated LDH and ferritin were characteristic. Due to low index of suspicion the diagnosis was delayed in majority of cases. Five of the 13 expired. Macrophage Activation Syndrome is associated with a high mortality and should be considered in the differential diagnosis of unexplained pancytopenia in-patients with connective tissue disease and malignancy.