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

Abstract

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.

• Authors: Nuray Uslu, Hulya Demir, Gunay Balta, Inci N Saltik-Temizel, Hasan Ozen, Figen Gürakan, Aysel Yüce
• Journal: Journal of Crohn's & colitis (J Crohns Colitis) Vol. 4 Issue 3 Pg. 341-4 (Sep 2010) ISSN: 1876-4479 [Electronic] England
• PMID: 21122524 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2009 European Crohn's and Colitis Organisation. Published by Elsevier B.V. All rights reserved.

Chemical References

• Immunosuppressive Agents

Topics

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