Although hemophagocytic lymphohistiocytosis (HLH) rarely occurs in patients with inflammatory bowel disease (IBD), when HLH is present, it often co-occurs with a known infection, malignancy, and/or the use of immunomodulatory therapy, according to study results published in Digestive Diseases and Sciences.

Due to the rarity and severity of HLH, prompt recognition and aggressive therapy are imperative in treating the syndrome.

A team of researchers conducted a systematic literature review and search of the FDA Adverse Events Reporting System (FAERS) database to identify case reports and case series of HLH in a population of patients with moderate-to-severe IBD. A total of 2486 abstracts and 182 FAERS reports were included in the analysis.

The research team found 58 HLH cases among patients with IBD treated with a biologic therapy; 52 of these cases occurred during a biologic therapy course. Of the patients with IBD (mean age, 26 years; 70% men), 82.8% had Crohn disease and 17.2% had ulcerative colitis. The mean duration of IBD was 7.0 years before patients received an HLH diagnosis.  


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Among 50 patients for whom outcomes were known, 15 died. The mean survival duration in patients for whom HLH was fatal was 21.2 days following diagnosis. Among patients who received rapid treatment for HLH, 70% survived.

At the time of diagnosis, patients with HLH either received induction treatment with biologics (34.5%), were treated with a biologic with maintenance therapy (51.7%), were not on biologics at the time of diagnosis (6.9%), or did not have a known treatment status (8.6%).

Exposure to the antitumor necrosis factor agent infliximab was reported in 70.7% of patients and 39.7% of patients had received adalimumab. Exposures to other therapies were also reported.

Over 80% of the patients in this study were found to have opportunistic infections or malignancies. Active Epstein-Barr infection was found with 15 cases of HLH. Other active infections that were reported included cytomegalovirus, histoplasmosis, and tuberculosis.

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“Risk factors may include younger age, male gender, presence of [Crohn] disease, and induction phase of treatment,” wrote the investigators. “Prompt recognition, identification of an underlying cause, and emergent treatment are required to prevent death. Our findings suggest that HLH should be especially considered during early treatment for active moderate-to-severe IBD,” the researchers concluded.

Reference

Coburn ES, Siegel CA, Winter M, Shah ED. Hemophagocytic lymphohistiocytosis occurring in inflammatory bowel disease: systematic review [published online April 16, 2020]. Dig Dis Sci. doi: 10.1007/s10620-020-06252-z