Abstract
BackgroundHemophagocytic 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.MethodsRetrospective, descriptive study of pediatric patients admitted to a high-complexity institution in Cali, Colombia between 2012 and 2019 with HLH diagnosis. Medical history review to complete an electronic database and a secondary, descriptive analysis was carried out. The study was approved by the Institutional Ethics Committee.ResultsTwenty-one patients were included. 52.4 % of the population was male with a median age of 9.3 years [IQR (3.0-13.7 years)]. More than half of patients (66.6 %) had viral disease at diagnosis, the most frequent being Epstein-Barr Virus (EBV) (52.3 %) and dengue (14.3 %). Three patients had confirmed gene mutations (G6PC3, XIAP, and UNC13D). 95 % of the patients were treated with the HLH 2004 protocol, half of them received incomplete protocol with intravenous immunoglobulin (IVIG) 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.ConclusionsHLH is a complex disease that requires multidisciplinary management with secondary HLH due to EBV infection being a common cause. There is increasing awareness of HLH diagnosis in developing countries such as Colombia which can offer earlier treatment options and better outcomes.
Highlights
Hemophagocytic lymphohistiocytosis (HLH) is an exaggerated inflammatory reaction secondary to a host’s inadequate immune response causing a self-perpetuating loop of altered regulation
The term HLH encompasses a wide range of disorders including primary HLH [which includes familial HLH (FHLH), familial erythrophagocytic lymphohistiocytosis] and secondary HLH [infection-associated hemophagocytic syndrome and autoimmune-associated macrophage activation syndrome (MAS)].[1, 3]
We aimed to determine the frequency of hemophagocytic syndrome and describe the demographic, clinical and outcome characteristics of pediatric patients who were hospitalized a high complexity institution in Cali, Colombia between 2012 and 2019
Summary
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. Hemophagocytic lymphohistiocytosis (HLH) is an exaggerated and ineffective inflammatory reaction secondary to a host’s inadequate immune system response causing. Signs and symptoms of HLH are nonspecific and usually compatible with other common diseases such as infections, tumors and rheumatological diseases.[4, 5] HLH should be included in the differential diagnosis of other clinical conditions such as: (1) fever of unknown origin, (2) hepatitis with coagulopathy (30 % of patients with HLH present an increase of transaminases above 100 U/L), (3) sepsis with multiple organic failure, (4) lymphocytic encephalitis.[6]. Other criteria have been tested including the H-score.[8, 9]
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