Hemophagocytic Lymphohistiocytosis Secondary to Disseminated Histoplasmosis

Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory condition resulting in erroneous activation of the immune system. Treatment is directed at the underlying pathology that prompts activation of the immune system and usually includes immunosuppressant therapy, including steroids, etoposide, or rituximab. However, the best treatment for patients with significant infection remains unclear. Few cases of HLH are associated with disseminated histoplasmosis, and there are no clear treatment guidelines in these cases. Due to the significant morbidity and mortality associated with HLH, further investigation is needed to identify the best treatment regimens.