Medical College of Wisconsin
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Association of Crohn's disease, thiopurines, and primary epstein-barr virus infection with hemophagocytic lymphohistiocytosis. J Pediatr 2011 Nov;159(5):808-12

Date

07/05/2011

Pubmed ID

21722918

Pubmed Central ID

PMC3191286

DOI

10.1016/j.jpeds.2011.04.045

Scopus ID

2-s2.0-80054753242 (requires institutional sign-in at Scopus site)   75 Citations

Abstract

OBJECTIVE: To assess the incidence of hemophagocytic lymphohistiocytosis (HLH) in a well-defined population of children with inflammatory bowel disease (IBD) and evaluate the common clinical and laboratory characteristics of individuals with IBD who developed HLH.

STUDY DESIGN: We conducted a retrospective study of all children who developed HLH over an 8-year period. The incidence of HLH in patients with IBD was calculated using US census data and a statewide project examining the epidemiology of pediatric IBD.

RESULTS: Among children in Wisconsin, 20 cases of HLH occurred during the study period; 5 cases occurred in children with IBD. Common characteristics include: Crohn's disease (CD), thiopurine administration, fever lasting more than 5 days, lymphadenopathy, splenomegaly, anemia, lymphopenia, and elevated serum triglycerides and ferritin. Of the patients, 4 had primary Epstein-Barr virus infections. The incidence of HLH among all children in Wisconsin was 1.5 per 100 000 per year. The risk was more than 100-fold greater for children with CD (P < .00001).

CONCLUSIONS: Pediatric patients with CD are at increased risk for developing HLH; primary Epstein-Barr virus infection and thiopurine administration may be risk factors.

Author List

Biank VF, Sheth MK, Talano J, Margolis D, Simpson P, Kugathasan S, Stephens M

Authors

David A. Margolis BA, MD Chair, Professor in the Pediatrics department at Medical College of Wisconsin
Pippa M. Simpson PhD Adjunct Professor in the Pediatrics department at Medical College of Wisconsin
Julie-An M. Talano MD Professor in the Pediatrics department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Adolescent
Anemia
Azathioprine
Crohn Disease
Epstein-Barr Virus Infections
Ferritins
Fever
Humans
Immunosuppressive Agents
Incidence
Lymphatic Diseases
Lymphohistiocytosis, Hemophagocytic
Lymphopenia
Mercaptopurine
Retrospective Studies
Splenomegaly
Triglycerides
Wisconsin