Distinct Laboratory and Clinical Features of Secondary Hemophagocytic Lymphohistiocytosis in Pediatric Visceral Leishmaniasis: A Retrospective Analysis of 127 Children in Andalusia, Spain (2004-2019).
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López Marcos María, Ruiz Sáez Beatriz, Vílchez Pérez Juan Salvador, Moreno Pérez David, Carazo Gallego Begoña, Falcón Neyra Lola, Goycochea Valdivia Walter Alfredo, Obando Santaella Ignacio, Lucena Soto José Manuel, Díaz Martos Julio, Santos Pérez Juan Luis, Rodríguez Benjumea Margarita, Angulo González de Lara Raquel, Sánchez Codez Mª Isabel, Peromingo Matute Estrella, Cruz Cañete Marta, Calviño Molinero Jennifer, Hurtado Mingo Ángela, Lendínez Molinos Francisco, Medina Claros Antonio F, Muñoz Vilches Mª José, Neth Olaf, Olbrich Peter, GAIP (Grupo Andaluz de Infectología e Inmunopatología Pediátrica) Network
Аннотация
METHODS: Medical records of children (<14 years) admitted with VL to 10 pediatric units in Andalusia (2004-2019) were retrospectively reviewed. VL diagnosis was based on clinical presentation, serology, microscopy and molecular methods. Diagnosis of secondary hemophagocytic lymphohistiocytosis (sHLH) was established using the hemophagocytic lymphohistiocytosis-2004 criteria.
RESULTS: A total of 127 patients were identified. Median age was 14.5 months; the main clinical presentations were fever and splenomegaly (95.3% each). Cytopenias were the most common laboratory abnormalities. Diagnostics as well as treatment regimens varied over time and the participating centers. Liposomal amphotericin B was prescribed in 97.6%; relapses as well as adverse events were rarely observed (3.1% each). Thirty-seven patients, diagnosed with sHLH required longer hospital admission (P = 0.001), an increased number of platelet (P < 0.006) and red blood cell (P = 0.0001) transfusions and pediatric intensive care unit admission (P = 0.007). Monocytopenia (P = 0.011) and high C-reactive protein levels (P = 0.031), variables not included in the hemophagocytic lymphohistiocytosis-2004 criteria, were associated with sHLH. One patient deceased in the context of the Leishmania infection.
CONCLUSIONS: We report data on the largest pediatric VL cohort from Europe, commonly associated with sHLH. Raised C-reactive protein levels and monocytopenia appear to be associated with sHLH. The latter may help to identify these patients and to guide decisions regarding need of additional supportive clinical care and immunomodulatory therapies. The observed high rate of heterogeneity in terms of diagnosis and management warrants the establishment of appropriate guidelines.