Pediatric Hemophagocytic lymphohistiocytosis: A rarely diagnosed entity in a developing country

Abstract

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is an exaggerated inflammatory reaction secondary to a host's inadequate immune response causing a self-perpetuating loop of altered regulation. Signs and symptoms of HLH are compatible with other common diseases and are nonspecific. Underdiagnosis makes it difficult to estimate the real incidence of HLH, especially in developing countries. Materials and Methods: Retrospective, descriptive study of hospitalized pediatric patients admitted to a high-complexity institution in Cali, Colombia between 2012 and 2019 with HLH diagnosis. Medical history review to complete a virtual database. A secondary descriptive analysis was carried out. The study was approved by the Institutional Ethics Committee. Results: Twenty-one patients were included. 52.4% of the population was male with a median age of 9.3 years [IQR (3.0-13.7years)]. More than half of patients (66.6%) had viral isolation at diagnosis, the most frequent being Epstein-Barr Virus (EBV) (52.3%) and dengue (14.3%). Three patients had gene mutations (LYST, XIAP, and UNC13D). Ninety-five percent of the patients were treated with the HLH 2004 protocol, half of them received incomplete protocol with IgIV at high doses and/or systemic steroids, while the other half received the complete protocol including etoposide and cyclosporine. More than three-fourths (76.2%) required admission to an ICU with a median stay of 14 days [IQR (11-37 days)] and a median hospital stay of 30 days [IQR (18-93 days)]. 14.3% (n = 3) of patients died. Conclusions: HLH is an underdiagnosed pathology that requires greater sensitization in developing countries in order to make early diagnoses and obtain better outcomes.

Síndrome hemofagocítico asociado a dengue grave en una institución pediátrica de Cali - Colombia

“…Los pacientes fueron manejados con inmunoglobulina, metilprednisolona y ciclosporina, excepto uno que no recibió inhibidor de la calcineurina, solo prednisolona e inmunoglobulina intravenosa y todos se recuperaron. En la literatura se ha descrito mortalidad variable, con buenos desenlaces si se realiza un diagnóstico y tratamiento oportuno 12,14 .…”