Classical Hodgkin’s Lymphoma Presenting with Hemophagocytosis

Hemophagocyticlymphohistiocytosis (HLH) is a hyper inflammatory disorder which results from immune dysfunction, either from primary immune deficiency or acquired failure of normal immune homeostasis. There is often phagocytosis by macrophages of blood cells, hence the name “hemophagocytosis” [1]. HLH may be inherited (known as familial hemophagocyticlymphohistiocytosis), or it may occur secondary to another disease process, most commonly infection with various pathogens or hematologic malignancy (Table 1), [2]. In 1991, diagnostic guidelines for HLH were first presented (Table 2), [3]. In January 2004, the diagnostic criteria for HLH were revised [4]. HLH is a rare condition, and the diagnostic guidelines have important implications for the medical management of patients. Without recognition, HLH is a deadly syndrome with an estimated mortality of 90% without appropriate treatment [5].