Cutaneous Gamma Delta T cell Lymphoma Leading to Hemophagocytic Lymphohistiocytosis that Presented as Posterior Reversible Encephalopathy Syndrome

Cutaneous Gamma Delta T cell Lymphoma (CGDTCL) is an extremely rare and aggressive disease arising from immature T cells with gamma delta T cell receptors. CGDTCL can result in a life-threatening syndrome called Hemophagocytic Lymphohistiocytosis (HLH). HLH is a syndrome of fevers, cytopenia and multiorgan failure resulting from excessive immune activation. Early diagnosis of HLH is vital as a delay in diagnosis/treatment may result in grave outcomes. Our patient presented with Posterior Reversible Encephalopathy Syndrome (a syndrome characterized by headache, confusion, seizures and visual disturbance) prior to the diagnosis of HLH.