Secondary Hemophagocytic Lymphohistiocytosis in a Young Hispanic Adult

Hemophagocytic lymphohistiocytosis (HLH) is a disease caused by a severe immune system reaction that involves an overwhelming inflammatory response with overproduction of cytokines and hemophagocytosis. HLH is classified as primary HLH or familial HLH (PHLH or FHLH) and secondary HLH. PHLH is due to mutations in several genes that regulate immune cells, while secondary HLH is triggered by a severe illness (viral infections or malignancies) that induce an excessive immune response that is difficult to control. We present a case of a young Hispanic adult female with a medical history of diabetes mellitus type 1 and hepatitis E that was diagnosed with HLH secondary to lymphoma caused by Epstein Barr virus infection. The patient was started on broad-spectrum antibiotics and steroid therapy; however, the patient succumbed to the disease. HLH is associated with high mortality, mainly because it is not a very common entity and patients usually present critically ill and deteriorate very fast. Immunosuppression and treatment of the underlying disorder is the target of the treatment of HLH, however, the prognosis remains poor.