Hemophagocytic syndrome in a child with severe Crohn's disease and familial Mediterranean fever

J Crohns Colitis. 2010 Sep;4(3):341-4. doi: 10.1016/j.crohns.2009.12.005. Epub 2010 Jan 6.


Hemophagocytic lymphohistiocytosis (HLH) is a rare, potentially fatal, severe condition of hyperinflammation caused by the uncontrolled proliferation of activated lymphocytes and histiocytes secreting high amounts of inflammatory cytokines. Here we report a fatal hemophagocytic syndrome in a 11-year-old boy with a diagnosis of both Crohn's disease receiving immunosuppressive therapy and familial Mediterranean fever. It is important to evaluate the patients with inflammatory bowel disease receiving immunosuppressive therapy presenting with unexplained fever, cytopenia, progression of organomegaly and biochemical changes for the investigation of HLH for diagnosis and treatment.

Publication types

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

MeSH terms

  • Child
  • Crohn Disease / complications
  • Crohn Disease / drug therapy
  • Familial Mediterranean Fever / complications*
  • Familial Mediterranean Fever / drug therapy
  • Fatal Outcome
  • Humans
  • Immunosuppression Therapy / adverse effects
  • Immunosuppressive Agents / adverse effects
  • Lymphohistiocytosis, Hemophagocytic / complications
  • Lymphohistiocytosis, Hemophagocytic / diagnosis*
  • Lymphohistiocytosis, Hemophagocytic / drug therapy
  • Lymphohistiocytosis, Hemophagocytic / immunology
  • Male


  • Immunosuppressive Agents

Supplementary concepts

  • Pediatric Crohn's disease