Original ArticleAssociation of Crohn’s Disease, Thiopurines, and Primary Epstein-Barr Virus Infection with Hemophagocytic Lymphohistiocytosis
Section snippets
Methods
We conducted a retrospective chart review of all individuals with IBD who developed HLH at Children’s Hospital of Wisconsin between January 2000 and June 2008. Cases of HLH were identified through a database maintained by the Children’s Hospital of Wisconsin Hematology/Oncology and Bone Marrow Transplant Department. Patients with HLH and a diagnosis of IBD were extracted for analysis.
The data extracted included patients’ demographics, presenting symptoms, medical and surgical records,
Results
Between January 2000 and June 2008, 20 cases of HLH were identified and 5 of those patients had IBD. According to the 2005-2007 US Census Bureau data, the Wisconsin population was 5.5 million persons, and 24% were younger than 19 years of age (1.3 million).19 Based on these figures, the overall incidence rate was 1.5 cases of HLH per 100 000 children. In the multicenter IBD epidemiology project, 992 children were documented to have IBD, and 5 cases of HLH occurred in this population.18 The
Discussion
The incidence of HLH treated at our institution appears to be similar to that reported in the general population, which ranges from 1 in 50 000 to 12 in 1 000 000.2, 7, 15, 22 The results in our pediatric population experiencing IBD suggests that there is more than a 100-fold greater risk for their developing HLH than exists in the general population. Possible explanations for this discrepancy are that the data assume that all pediatric patients diagnosed with IBD were included in our
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2019, Clinical Gastroenterology and HepatologyCitation Excerpt :At an individual level, thiopurine-associated fatal infections have been reported mainly in young patients. These infections include severe forms of varicella20 and primary Epstein-Barr virus or cytomegalovirus infections complicated with hemophagocytic lymphohistiocytosis.21 In patients exposed to anti-TNF agents, fatal cases of infections are related to various types of opportunistic infections.16
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Supported by NIH/NIGMS (5K08GM077395 to M. Stephens). M. Stephens has received research grants from the Centocor Inc, Abbott Laboratories, and Schwartz Biosciences. All other authors declare no conflicts of interest.