Histiocytic haemophagocytosis in a patient with Kawasaki disease: changes in the hypercytokinaemic state

Eur J Pediatr. 1995 Jul;154(7):539-41. doi: 10.1007/BF02074830.


A 32-month-old Japanese boy exhibited haemophagocytic syndrome (HPS) during the recurrent course of Kawasaki disease. Despite repeated gamma-globulin therapy, he developed cytopenia with marked hepatomegaly and evidence of histiocytic haemophagocytosis in the bone marrow. Serum levels of interferon-gamma and tumour necrosis factor, but not of interleukin-1 beta, increased in parallel with his symptoms. No confirmation was obtained of the association of toxic reactions to the used drugs. No coronary lesions remained as sequelae.

Conclusion: Cytopenia in Kawasaki disease could herald HPS, and the hypercytokinaemia involved in the two febrile syndromes might be of distinct nature.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Child, Preschool
  • Cytokines / blood*
  • Histiocytosis, Non-Langerhans-Cell / immunology*
  • Humans
  • Interferon-gamma / blood
  • Interleukin-1 / blood
  • Lymphocyte Activation / immunology
  • Male
  • Mucocutaneous Lymph Node Syndrome / immunology*
  • Recurrence
  • T-Lymphocytes / immunology
  • Tumor Necrosis Factor-alpha / metabolism


  • Cytokines
  • Interleukin-1
  • Tumor Necrosis Factor-alpha
  • Interferon-gamma