Systemic lupus erythematosus (SLE) is a multisystemic chronic autoimmune disease with a varied clinical history. Hematological involvement is common, and it has been linked to an increase in morbidity and mortality, as well as an increased risk of infection and lower survival rates. The goal of this study was to describe the hematologic changes in paediatric SLE patients when they were first diagnosed. Methods. A retrospective descriptive analysis was undertaken on patients with SLE who were treated at the Exequiel Gonzalez Cortes Children's Hospital's Immunorheumatology Outpatients Center in Santiago, Chile. Thirty-three clinical files were examined, and demographic and clinical information was collected. Results. Thirty patients met the criteria for inclusion, 22 of them were females with an average age of 11.6 years (range 5 to 14). Hematological compromise was seen in 60% of the participants (n = 18). Lympopenia was the most common haematological abnormality, with 14 individuals, followed by autoimmune hemolytic anaemia (HA) with 11 patients. Ten individuals had leukopenia, while three had thrombocytopenia. Lympopenia was found to be associated with leukopenia in 50% of the patients (n = 9) while HA was shown to be associated with lymphopenia in 8 individuals. Skin involvement was the most common extra-hematological manifestation in patients with haematological changes (26/30), followed by kidney involvement (25), articular involvement (22), and serositis (17).
Conclusions. In this cohort of paediatric SLE cases, haematological involvement was common, and this is accounted for in both classic and contemporary diagnostic criteria. It can be the first symptom in a group of patients, necessitating a differential diagnosis and close monitoring. In other circumstances, it could be a symptom of severe SLE that necessitates aggressive treatment.Author(s) Details
María-Soledad Ayala
Department of Pediatrics School of Medicine University of Chile, Santiago, Chile.
Ximena Norambuena
Immunorheumatology Unit Children´s Hospital Dr. Exequiel Gonzalez Cortes, Santiago, Chile and Pediatric Rheumatology International Trials Organization (PRINTO) Member, Chile.
Alfonso Loosli
Immunorheumatology Unit Children´s Hospital Dr. Exequiel Gonzalez Cortes, Santiago, Chile.
Arnoldo Quezada
Department of Pediatrics School of Medicine University of Chile, Santiago, Chile and Pediatric Rheumatology International Trials Organization (PRINTO) Member, Chile.
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