Diagnosing Primary Hemophagocytic Lymphohistiocytosis in an Adult: A Case Report

Primary hemophagocytic lymphohistiocytosis (HLH) is a rare, often fatal immune disorder characterized by an overactivation of the immune system. This disease is more common in children but has been known to occur in the occasional adult. The criteria for diagnosis in children do not correlate well with diagnosis in adults, and the numerous variations of presentation in adults often lead to a delay in diagnosis and treatment initiation. This case report highlights an example of primary HLH in a 41-year-old female patient who experienced a delay in diagnosis and appropriate treatment due to the similarity of her early symptoms to more common conditions. Delays in diagnosis and treatment can lead to poor outcomes, including death. Thus, it is important for providers to be able to recognize the varying signs and symptoms of primary HLH in an adult to ensure treatment is initiated as early as possible in the disease course.