1 - Pediatria Médica, Área de Pediatria, Hospital Dona Estefânia, Unidade Local de Saúde de São José
2 - Infeciologia Pediátrica, Área de Pediatria, Hospital Dona Estefânia, Unidade Local de Saúde de São José
3 - Hematologia Pediátrica, Área de Pediatria, Hospital Dona Estefânia, Unidade Local de Saúde de São José
4 - Imunodeficiências Primárias, Área de Pediatria, Hospital Dona Estefânia, Unidade Local de Saúde de São José
- Publicação em versão integral
Resumo:
Introdução: Autoimmune hemolytic anemia (AIHA) and hemophagocytic lymphohistiocytosis (HLH) are rare complications of infectious mononucleosis. Epstein–Barr virus-associated AIHA occurs in only 0.5-3% of hospitalized patients.
Relato de caso: The authors describe a 12-year-old male with acute infectious mononucleosis, hepatitis, cholestasis, and an autoimmune hemolytic disorder caused by cold agglutinins IgM (anti-i specificity). Clinical deterioration with persistent fever, anemia, and hepatosplenomegaly was consistent with cold AIHA plus concomitant HLH. The patient was treated with corticosteroids and acyclovir, with an uneventfully recovery.
Conclusão: Although rare, cold agglutinin syndrome and HLH can complicate infectious mononucleosis and should be considered in a patient with clinical deterioration. Corticosteroids are the mainstay treatment of HLH and may be beneficial in infection-associated cold agglutinin syndrome.
Palavras Chave: Autoimmune hemolytic anemia; Epstein–Barr virus; hemophagocytic syndrome; infectious mononucleosis