LINFOHISTIOCITOSE HEMOFAGOCÍTICA FATAL EM CRIANÇA: DA APRESENTAÇÃO INESPECÍFICA AO DESFECHO LETAL
Luísa Lyrio Stábille, Rafael Cardoso Barbosa, Stella Vasques Resende, Vanessa Silva Lima, Isabela Garcia Ismael, Kellen Cristina Kamimura Barbosa Silva
Resumo
Introdução: A síndrome hemofagocítica (SHF) é uma condição hiperinflamatória rara e potencialmente fatal, caracterizada por ativação descontrolada do sistema imunológico. Apresentação do Caso: Este relato descreve o caso de um paciente masculino de 6 anos, previamente hígido, admitido com febre persistente, linfonodomegalia cervical e prurido difuso. Na admissão, apresentava desidratação grave, taquicardia, pancitopenia (hemoglobina 7,5 g/dL, plaquetas 33.000/mm³), coagulopatia, hipofibrinogenemia (188 mg/dL) e hiperferritinemia marcante (3487,91 ng/mL). A investigação inicial considerou múltiplas hipóteses diagnósticas, incluindo infecções endêmicas e neoplasias hematológicas. O paciente evoluiu com instabilidade hemodinâmica, necessitando transferência para UTI pediátrica, onde foram iniciados antibióticos de amplo espectro, suporte vasoativo e terapia imunossupressora. Desenvolveu comprometimento cardíaco grave (fração de ejeção 30%), insuficiência renal aguda e coagulopatia refratária. Considerações Finais: Apesar do suporte intensivo multidisciplinar, evoluiu com falência de múltiplos órgãos e óbito após cinco dias de internação. A confirmação necroscópica de SHF reforça a importância do reconhecimento precoce desta síndrome em pacientes pediátricos com febre persistente, citopenias e hiperferritinemia, destacando a necessidade de alto índice de suspeição clínica e instituição imediata de terapia imunossupressora.
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