CMV Induced HLH in a Patient with Granulomatosis with Polyangiitis

Abstract

Hemophagocytic lymphohistiocytosis [HLH] is a rare condition marked by over-activation of the immune system leading to uncontrolled systemic inflammation. It is characterized by cytokine storm with over-activation of T-cells and macrophages, leading to tissue damage, multi-organ failure [1]. HLH presents a wide array of symptoms that are not easily differentiated from systemic inflammatory response and as such the diagnosis is often delayed. Mortality rates are high, ranging from 40%-70% [2]. To confirm the diagnosis, the HLH-2004 criteria are used. The criteria include molecular diagnosis and/or clinical and laboratory diagnosis [1]. HLH can be associated with rheumatologic conditions and triggered by infection. In this case report we highlight a middle-aged woman with a rare cause of HLH: cytomegalovirus-induced HLH in a patient with granulomatosis with polyangiitis.