A Rare Case of Macrophage Activation Syndrome in a Patient with Behcet’s Disease

Abstract

A 37-year-old male of Pakistani origin with longstanding fever developed orogenital ulcers, skin rash, cellulitis and thrombophlebitis had a positive HLAB51 and was diagnosed with Behcet’s disease. During the same hospital stay patient acutely developed pancytopenia, non-remitting fever, a high serum triglycerides and serum ferritin and bone marrow biopsy evidence of Macrophage activation syndrome (MAS), but remained non responsive to intravenous steroids and intolerant to intravenous immunoglobulins (IVIG) and finally succumbed to septic shock.