Features of patients with inflammatory bowel diseases who develop hemophagocytic lymphohistiocytosis

Li, Y.; Li, C. F.; Zhang, J.; Xia, Xuefeng; Zhou, Liya; Liu, J. J.; Song, Zhiqiang; Lv, Yiran; Wang, A. Y.; Zhang, Y. P.; Liang, Chao; Shi, Yanhong; Quigley, Eamonn M.M.; Huang, Yonghui; Ding, Shi Gang

doi:10.1007/s00384-015-2485-2

Cited by 6 publications

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“…It can be frequently mistaken to an exacerbation of a previously diagnosed disease, such as IBD, and hence it can show similar symptoms, such as diarrhea and gastrointestinal bleeding, as presented in Table 1 [42]. However, IBD associated with HLH is rare [43].…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

“…Prolonged courses of thiopurines may decrease circulating lymphocytes due to their effect of apoptosis, which is important to HLH [43]. However, they also affect immunological defense, which leads to a predisposition to a viral infection, such as CMV, EBV or varicella zoster virus [43]. The most common clinical features are fever, hepatosplenomegaly, lymphadenopathy, neurologic symptoms (seizures, ataxia, mental status changes and headache) and skin rash [42].…”

Section: Discussionmentioning

confidence: 99%

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Hemophagocytic Lymphohistiocytosis and Inflammatory Bowel Disease: Case Report and Systematic Review

et al. 2020

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Aim: To report a case of a female patient with hemophagocytic lymphohistiocytosis (HLH) and to systematically review the available cases of the association between HLH and inflammatory bowel disease (IBD). Methods: In accordance to Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines, retrieval of studies was based on Medical Subject Headings and Health Sciences Descriptors, which were combined using Boolean operators. Searches were run on the electronic databases Scopus, Web of Science, MED-LINE (PubMed), Biblioteca Regional de Medicina, Latin American and Caribbean Health Sciences Literature, Cochrane Library for Systematic Reviews and Opengrey.eu. Languages were restricted to English, Spanish and Portuguese. There was no date of publication restrictions. The reference lists of the studies retrieved were searched manually. Results: The search strategy retrieved 223 references. In the final analysis, 28 references were included, with the report of 35 cases. The most common clinical finding was fever, 57% of patients had a cytomegalovirus infection and 30 patients were on thiopurines previously to HLH diagnosis. Most patients were treated with steroids and antiviral therapy. All-cause mortality was 22%. Conclusion: These findings suggest that there might be a connection of HLH to IBD, opportunistic viral infections and the use of thiopurines. Due to the severity of such disease, the clinical suspicion is paramount to early diagnosis and therapy.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Hemophagocytic Lymphohistiocytosis and Inflammatory Bowel Disease: Case Report and Systematic Review

et al. 2020

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“…In fact, opportunistic infection or malignancy was found in more than 80% of all known IBD patients with at least some biologic exposure who developed HLH in a recent systematic review [18]. Studies reporting HLH occurring in IBD show a more frequent occurrence of HLH in patients with Crohn's disease compared to UC [18,19], yet the reason behind this susceptibility is not known. In this particular patient, in addition to biologic therapy with vedolizumab, genetic predisposition was another concern raised upon this second episode of HLH, once presentation at a late age, even in adulthood, can occur [20].…”

Section: Discussionmentioning

confidence: 99%

Persistent Fever after COVID-19 Vaccination in a Patient with Ulcerative Colitis: A Call for Attention

Rodrigues¹,

Carvalho²,

Ventura³

et al. 2023

GE Port J Gastroenterol

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The development of vaccinations has been game-changing in the ongoing effort to combat the COVID-19 pandemic. Until now, adverse effects are being reported at low frequency, including thrombocytopenia and myocarditis. Careful monitoring for any suspicious symptoms and signs following vaccination is necessary. We report a case of hemophagocytic lymphohistiocytosis (HLH) after mRNA COVID-19 vaccine in a 23-year-old female with ulcerative colitis. Diagnosis was made according to HLH-2004 criteria and the patient was treated with dexamethasone with response. Our report aimed to draw attention to the potential relation between COVID-19 vaccines and HLH and the necessity of continued surveillance, especially in at-risk populations such as those with underlying immune dysregulation.

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“…Therefore, immunosuppressive therapy can impair a patient’s ability to eliminate EBV infection, leading to severe infections and complications, including the development and exacerbation of HLH. For example, patients with inflammatory bowel disease undergoing thiopurine therapy carry an increased risk for virus-induced HLH, the majority of which are due to primary EBV and CMV infections [ 38 ]. Several cases of EBV-HLH due to reactivation of EBV during immunosuppressive treatment for rheumatologic diseases have also been reported [ 39 , 40 ].…”

Section: Discussionmentioning

confidence: 99%

Spontaneous Recovery of Hemophagocytic Lymphohistiocytosis Due to Primary Epstein-Barr Virus Infection in an Adult Patient

Matsuo¹,

Iwanami²,

Hiraoka³

et al. 2021

Am J Case Rep

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Patient: Male, 34-year-old Final Diagnosis: Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis Symptoms: Fever • rash Medication: — Clinical Procedure: — Specialty: Hematology • Immunology • Infectious Diseases Objective: Unusual clinical course Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening systemic hyperinflammatory condition. Most adult HLH cases are secondary to infection, malignancy, and rheumatic diseases. Epstein-Barr virus (EBV) infection is the most frequent cause of infection-induced HLH. Early treatment with dexamethasone, etopo-side, and cyclosporine is generally recommended for adult patients with HLH. However, this treatment regimen was established based on pediatric clinical trial data; thus, its efficacy and validity in adults remain unclear. Because little is known about the disease course of untreated adult EBV-associated HLH (EBV-HLH), we report a case of an adult patient who recovered from EBV-HLH spontaneously without specific treatment and discuss potential treatment strategies. Case Report: A 34-year-old man presented to the emergency department with a 7-day history of fever, headache, and sore throat. The main laboratory test abnormalities were elevated liver enzymes, hyperbilirubinemia, hypertriglyceridemia, and hyperferritinemia. Serologic tests confirmed acute primary EBV infection. He was diagnosed with EBV-HLH based on the HLH-2004 diagnostic criteria and the HLH probability calculator (HScore). Because he was clinically stable, we did not initiate immunosuppressive/cytotoxic treatment targeting HLH. High-grade fever persisted, but the abnormalities in his laboratory data improved spontaneously, and he did not develop major organ failure. His fever resolved on day 29 without HLH-specific treatment. Conclusions: In clinically stable adult patients with EBV-HLH without major organ failure, it might be an acceptable alternative to observe the patient for several weeks before initiating HLH-specific treatment. Further research is required to better predict the subset of patients who can be safely observed without treatment.

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Features of patients with inflammatory bowel diseases who develop hemophagocytic lymphohistiocytosis

Cited by 6 publications

References 0 publications

Hemophagocytic Lymphohistiocytosis and Inflammatory Bowel Disease: Case Report and Systematic Review

Hemophagocytic Lymphohistiocytosis and Inflammatory Bowel Disease: Case Report and Systematic Review

Persistent Fever after COVID-19 Vaccination in a Patient with Ulcerative Colitis: A Call for Attention

Spontaneous Recovery of Hemophagocytic Lymphohistiocytosis Due to Primary Epstein-Barr Virus Infection in an Adult Patient

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