A Case Report of Aggressive Post-Infectious Hemophagocytic Lymphohistiocytosis in an Immunocompetent Adult

Cureus. 2023 Dec 30;15(12):e51334. doi: 10.7759/cureus.51334. eCollection 2023 Dec.

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is an acute inflammatory syndrome triggered by immune events such as infections, inflammation, autoimmune diseases, and malignancies. Initial presentations can range from vague symptoms to infectious features such as fever. Given its aggressive nature, timely diagnosis and immediate treatment are crucial to achieving optimal patient outcomes. Recently, the HLH score (HScore) criteria have been applied as diagnostic criteria, offering a broader scope compared to the previous HLH-2004 score, which was primarily based on pediatric populations. The standard treatment for decades has involved the combination of etoposide and high-dose steroids, and it is recommended to initiate treatment as soon as possible, even in the absence of a bone marrow test or when there is suspicion of the diagnosis. In this case presentation, we aim to underscore the significance of maintaining a high level of suspicion for HLH and the importance of promptly initiating treatment.

Keywords: diagnosis criteria; etoposide; hemophagocytic lymphohistiocytosis (hlh); hscore; timely treatment.

Publication types

  • Case Reports