Perfil clínico, biomarcadores de la Histiocitosis de Células de Langerhans y su respuesta al tratamiento en pacientes diagnosticados en Solca-Guayaquil

Abstract

Langerhans Cell Histiocytosis (LCH) is a myeloid-inflammatory disease that presents with a variety of clinical manifestations, ranging from unifocal to multifocal lesions in a single organ, to multisystem involvement. The identification of biomarkers such as CD1A, CD207, and S-100, and the detection of mutations in BRAFV600E, are crucial for the diagnosis of LCH. However, in Ecuador, the distribution of LCH patients according to clinical profile and biomarkers is not well documented, which limits the understanding of the disease's progression and treatment effectiveness. This retrospective study analyzed 42 patients diagnosed with LCH from the SOLCA-Guayaquil database over the past five years (2018-2022), with the aim of describing their clinical characteristics, biomarkers, and evaluating the relationship with treatment response. The majority of patients were male, aged between 5 and 10 years. All patients showed bone involvement and received triple therapy, with a partial response to treatment in 52.38% of cases. The most frequent biomarkers were S-100, CD1A, and CD68, respectively. The results indicated that CD1A and CD68 were significantly associated with a partial response to treatment (p=0.089 and p=0.044, respectively). No substantial relationship was found between the clinical profile, the presence of biomarkers, and the overall treatment response. These findings highlight the importance of continuing to investigate the use of biomarkers and mutations for the development of personalized strategies in the management and follow-up of LCH. Key words: Langerhans Cell Histiocytosis,