9/17/2023 0 Comments Hlh up to date![]() Haemophagocytic lymphohistiocytosis in inflammatory bowel disease with virus infection. Li Y, Xia X, Zhang J, Song Z, Zhou L, Zhang Y, et al. Infection is the major trigger of hemophagocytic syndrome in adult patients treated with biological therapies. Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement. Moher D, Liberati A, Tetzlaff J, Altman DG, PRISMA Group. Risk of Malignant Cancers in Inflammatory Bowel Disease. Biologic Therapies and Risk of Infection and Malignancy in Patients With Inflammatory Bowel Disease: A Systematic Review and Network Meta-analysis. Available from: īonovas S, Fiorino G, Allocca M, et al. How I treat hemophagocytic lymphohistiocytosis in the adult patient | Blood Journal. Hemophagocytic lymphohistiocytosis: review of etiologies and management. Recommendations for the management of hemophagocytic lymphohistiocytosis in adults. Our study is not intended to assess risk of HLH with specific IBD therapies. Risk factors may include younger age, male gender, presence of Crohn’s disease, and induction phase of treatment. Most cases had an identifiable infection or malignancy at the time of diagnosis as well as history of immunomodulator use. HLH is rare among IBD patients exposed to biologic therapy. ![]() Five patients restarted biologics after HLH resolved, and one patient developed recurrent HLH. Survival was 70% if promptly recognized and treated. Underlying opportunistic infection or lymphoma was found in > 80% of cases. 2/3 of cases did not report prior AZA/6MP exposure. All cases occurred on patients exposed to anti-TNF agents, but cases with anti-integrin or anti-IL-12/23 exposure were reported. ![]() 34.5% of patients were undergoing induction therapy at HLH diagnosis. Resultsĥ8 cases of HLH occurring in IBD patients are known (mean age: 26.0 years, 70% male, 83% with Crohn’s disease, mean disease duration 7.0 years). ![]() Use of biologics was used as a surrogate definition for disease severity (consistent with usual and contemporary clinical management), to enable identification of rare HLH cases with the highest fidelity. We performed a systematic review of the literature (PubMED EMBASE) and FDA Adverse Event Reporting System in accordance with the PRISMA statement. We aimed to systematically characterize HLH in moderate-to-severe inflammatory bowel disease (IBD). Hemophagocytic lymphohistiocytosis (HLH) is a rare and aggressive syndrome of excessive cytokine requiring prompt recognition and aggressive therapy. ![]()
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