Kawasaki disease preceding haemophagocytic lymphohistiocytosis: challenges for developing world practitioners

Pediatr Blood Cancer. 2010 Jul 1;54(7):1023-5. doi: 10.1002/pbc.22422.


Kawasaki disease (KD) is a recognised precipitant of haemophagocytic lymphohistiocytosis (HLH). Although KD has been previously described in the developing world, there are no reported cases of KD preceding HLH. We report a case of a child with a persistent rash and unremitting fever consistent with the diagnosis of KD, who was found to have HLH, after intravenous gamma globulin failed to produce a clinical response. The diagnosis was made using the revised diagnostic criteria for HLH from the Histiocyte Society (1994). She fulfilled six of the eight clinical and laboratory criteria needed to make the diagnosis.

MeSH terms

  • Anti-Bacterial Agents / therapeutic use
  • Anti-Inflammatory Agents / therapeutic use
  • Ceftriaxone / therapeutic use
  • Child
  • Developing Countries
  • Dexamethasone / therapeutic use
  • Humans
  • Immunologic Factors / therapeutic use
  • Lymphohistiocytosis, Hemophagocytic / drug therapy
  • Lymphohistiocytosis, Hemophagocytic / etiology*
  • Lymphohistiocytosis, Hemophagocytic / physiopathology
  • Mucocutaneous Lymph Node Syndrome / complications*
  • Mucocutaneous Lymph Node Syndrome / drug therapy
  • Mucocutaneous Lymph Node Syndrome / physiopathology
  • gamma-Globulins / therapeutic use


  • Anti-Bacterial Agents
  • Anti-Inflammatory Agents
  • Immunologic Factors
  • gamma-Globulins
  • Ceftriaxone
  • Dexamethasone