O DESAFIO DIAGNÓSTICO DA LINFOHISTIOCITOSE HEMOFAGOCÍTICA NO PACIENTE PEDIÁTRICO GRAVE

Introduction: this case report details the management of a 7-year-old patient with a history of neonatal hypoglycemia, biliary lithiasis, and short stature, who was admitted with pulmonary sepsis that progressed to septic shock and severe cardiovascular dysfunction. The diagnoses included multisystem inflammatory syndrome (MISC) and macrophage activation syndrome (MAS), with positive COVID-19. Methodology: data were extracted from the patient’s medical records, involving a thorough review from admission to discharge. The methodology included analyzing laboratory tests, imaging results, and documenting therapeutic interventions. The case was described chronologically, detailing clinical progression and response to administered therapies. Key interventions included the use of vasopressors (dobutamine, adrenaline, noradrenaline, and milrinone), hydrocortisone, immunoglobulin, and methylprednisolone. The analysis focused on the impact of these therapies on the patient’s condition and the need for ongoing adjustments in treatment. Conclusions: this report illustrates the management of a complex pediatric case involving pulmonary sepsis, MISC, MAS, and hemophagocytic lymphohistiocytosis (HLH). The experience underscores the complexity of treating severe and rare conditions in pediatrics. The administration of immunoglobulin and methylprednisolone was crucial in the initial management, helping to stabilize cardiovascular function and improve the inflammatory response. Continuous monitoring of cardiovascular function and inflammatory markers was essential for adjusting interventions and optimizing treatment. The case highlights the importance of a comprehensive therapeutic approach and rigorous follow-up to address multiple facets of critical conditions. Collaboration among medical specialties and the application of specific protocols were fundamental to the success of the treatment and the patient’s recovery.