Hemophagocytic Lymphohistiocytosis in the Setting of Disseminated Histoplasmosis and Uncontrolled HIV

Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threateningly aggressive syndrome caused by excessive immune activation. It involves the abnormal activation of lymphocytes and macrophages which leads to tissue destruction and inflammation. Traditionally HLH classification is currently separated into primary and secondary HLH based on genetic versus nongenetic events such as infection, malignancy, or autoimmune disorders. In this case report, we present the case of a middle-aged woman presenting with HIV with medication noncompliance who presented to the emergency department with pancytopenia as well as disseminated histoplasmosis and was diagnosed with HLH based on the HLH-2004 guidelines and treated in accordance with the HLH-94 protocol. The patient also underwent treatment for the management of her histoplasmosis with a favorable outcome. This case demonstrates that HLH is best treated through management of the underlying process that triggered the syndrome such as infection as in this patient in addition to management per HLH-94 protocol early on in the course of the disease in order to have the best chance at a positive clinical outcome.