Association of Crohn¡¯s Disease, Thiopurines, and Primary Epstein-Barr Virus Infection with Hemophagocytic Lymphohistiocytosis

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• 作者：Vincent F. Biank ; MD^a ; Mehul K. Sheth ; DO^a ; Julie Talano ; MD^b ; David Margolis ; MD^b ; Pippa Simpson ; PhD^c ; Subra Kugathasan ; MD^d ; Michael Stephens ; MD^a ; ^{mstephen@mcw.edu}
• 关键词：CD ; Crohn's disease ; EBNA ; Epstein-Barr nuclear antigen ; EBV ; Epstein-Barr Virus ; HLH ; Hemophagocytic lymphohistiocytosis ; IBD ; Inflammatory bowel disease
• 刊名：The Journal of Pediatrics
• 出版年：2011
• 出版时间：November, 2011
• 年：2011
• 卷：159
• 期：5
• 页码：808-812
• 全文大小：124 K

文摘

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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.