Abstract

Rationale and Objectives Hemophagocytic lymphohistiocytosis (HLH) is a rare and fatal syndrome characterized by uncontrolled immune response leading to excessive inflammation and end-organ damage. This study investigates the presentation and treatment outcomes of adult patients with HLH in a low-resource setting.

Research Design and Methodology A retrospective and descriptive chart review of 25 adult patients diagnosed with HLH from January 2016 to December 2022.

Results The median age of the patients was 31 years (IQR: 25-46 years), and more than half (56%) were male. The most common etiology for HLH is idiopathic, followed by malignancy-associated, and the rest were either infection-associated or autoimmune-associated. The most frequent presenting signs and symptoms were high-grade fever, cachexia, and hepatosplenomegaly. All patients exhibited anemia, thrombocytopenia, elevated ferritin levels, elevated LDH, and triglyceridemia. In terms of outcomes, acute respiratory failure and septic shock were the most common causes of death. The majority of patients died (84%) in the study.

Discussion and Conclusion HLH presents with nonspecific symptoms; thus, a high level of clinical suspicion is paramount in the diagnosis. Furthermore, aggressive supportive measures, elimination of HLH triggers, and suppression of the aberrant inflammatory response are equally important in the management of HLH.

Key Words: Hemophagocytic lymphohistiocytosis, HLH, hemophagocytic syndrome, Filipino, treatment outcomes