Hemophagocytic lymphohistiocytosis in pediatrics: diagnostic and therapeutic challenges in a tertiary hospital in Guayaquil, Ecuador.

Abstract

In pediatrics, the secondary form is the most prevalent and is usually triggered by infections, malignancies, or autoimmune diseases. Due to its rapid progression and high mortality rate, early diagnosis is crucial for survival. Objective. To describe the clinical characteristics, laboratory findings, etiology, treatment, and outcomes of pediatric patients with HLH at a tertiary care hospital in Guayaquil, Ecuador. Materials and methods. A descriptive and retrospective study of a series of 22 cases (January 2019–September 2025) diagnosed according to the HLH-2004 criteria. Demographic variables, paraclinical findings, bone marrow studies, infectious triggers, and treatment protocols were analyzed. Results. The mean age was 4.26 years. Fever was universal (100%), and splenomegaly affected 86.36% of patients. Universal hyperferritinemia (mean: 8,804 ng/mL) and hypofibrinogenemia were observed in 86.36% of cases. Viral infections were the main trigger (86.36%), with dengue and SARS-CoV-2 being the most prevalent (40.91% each), along with a high incidence of multisystem inflammatory syndrome (MIS-C) in 54.55%. Treatment included dexamethasone (90.91%), immunoglobulin (54.55%), and etoposide (18.18%). Mortality was 22.73%. Discussion. Pediatric HLH presents with high morbidity and is frequently associated with endemic and emerging viruses in the region. Diagnosis based on biomarkers such as ferritin is crucial, especially in severe cases of dengue or MIS-C, to initiate aggressive immunosuppressive therapy.