[Hemophagocytic syndrome associated with Hodgkin lymphoma and Epstein-Barr virus infection. A case report]

Rev Alerg Mex. 2020 Apr-Jun;67(2):183-188. doi: 10.29262/ram.v67i2.765.
[Article in Spanish]

Abstract

Background: Hodgkin lymphoma is a malignant neoplasm of B lymphoid cells whose histologic characteristic is the presence of Reed-Sternberg cells in an inflammatory environment.

Case report: A 37-year-old woman with a history of up to 40°C fever for four months, progressive and bilateral decrease in hearing acuity, weight loss of up to 6 kg, cervical lymphadenopathy, hepatosplenomegaly, and pancytopenia. Auditory sensory neuropathy was confirmed. The patient developed hemophagocytic syndrome, therefore, infectious and autoimmune etiologies were ruled out. The CT scan revealed hepatosplenomegaly with thoracic and abdominal cervical nodes, with loss of fatty hilum. The laboratory tests showed evidence that suggested the reactivation of the Epstein-Barr virus. Through a submandibular node biopsy, the diagnostic conclusion was that lymphocyte-rich classical Hodgkin's lymphoma was present.

Conclusion: This is the first report in Latin American literature about a patient with hemophagocytic syndrome that is secondary to classic Hodgkin lymphoma and associated with Epstein-Barr infection.

Antecedentes: El linfoma de Hodgkin es una neoplasia maligna de células linfoides tipo B cuya característica histológica es la presencia de células de Reed-Sternberg en un medio inflamatorio. Caso clínico: Mujer de 37 años, con fiebre de hasta 40 °C desde cuatro meses atrás, disminución bilateral y progresiva de la agudeza auditiva, pérdida ponderal de 6 kg, linfadenopatía cervical, hepatoesplenomegalia y pancitopenia. Se corroboró neuropatía sensorial auditiva. La paciente desarrolló síndrome hemofagocítico, por lo que se descartaron procesos infecciosos o autoinmunes. La tomografía reveló hepatoesplenomegalia, ganglios cervicales torácicos y abdominales con pérdida del hilio graso; en los estudios de laboratorio se evidenciaron datos sugerentes de reactivación del virus de Epstein-Barr. Mediante biopsia de ganglio submandibular se concluyó que se trataba de linfoma de Hodgkin tipo clásico rico en linfocitos. Conclusión: La paciente descrita con síndrome hemofagocítico secundario a linfoma de Hodgkin clásico asociado a infección por Epstein-Barr constituye el primero caso reportado en la literatura latinoamericana.

Keywords: Epstein-Barr virus; Hemophagocytic syndrome; Lymphocyte-rich Hodgkin lymphoma.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Epstein-Barr Virus Infections / complications*
  • Female
  • Hodgkin Disease / complications*
  • Humans
  • Lymphohistiocytosis, Hemophagocytic / etiology*