Infection is the major trigger of hemophagocytic syndrome in adult patients treated with biological therapies

Brito-Zerón, Pilar; Bosch, Xavier; Pérez-de-Lis, M.; Pérez-Álvarez, R.; Fraile, G.; Gheitasi, H.; Retamozo, Soledad; Bové, Albert; Monclús, Ester; Escoda, Ona; Moreno, Asunción; López‐Guillermo, Armando; Khamashta, Munther A.; Ramos‐Casals, Manuel

doi:10.1016/j.semarthrit.2015.07.004

Cited by 55 publications

(38 citation statements)

References 44 publications

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“…The importance of primary EBV infection in patients with IBD is associated with the possible development of serious complications, such as hemophagocytic syndrome and lymphoma, in those receiving treatment with thiopurines . The association between hemophagocytic syndrome and anti‐TNF drugs is unclear; thus, 29 adult patients underwent biological treatment, five of them had IBD, developed hemophagocytic syndrome, some of them received non‐anti‐TNF biological therapy and others additionally received immunomodulators . In contrast, a study on paediatric IBD did not find any association with the use of biological drugs .…”

Section: Discussionmentioning

confidence: 97%

Impact of Epstein‐Barr virus serological status on clinical outcomes in adult patients with inflammatory bowel disease

Francisco

Castaño-García

Martínez-González

et al. 2018

Aliment Pharmacol Ther

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Section: Discussionmentioning

confidence: 97%

Impact of Epstein‐Barr virus serological status on clinical outcomes in adult patients with inflammatory bowel disease

Francisco

Castaño-García

Martínez-González

et al. 2018

Aliment Pharmacol Ther

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“…A few studies have focused on the role of acute infectious triggers in HLH survival, mainly in pediatric patients, 20 , 21 but also in adults treated with biological agents. 22 However, some descriptive studies have reported higher mortality rates in patients presenting with bacterial infections 13 , 23 , 24 or co-infections. 21 , 25 Our study reports, for the first time, that both the variety and number of infectious triggers implicated in HLH have a considerable influence on survival using time-to-event statistical models; the best survival curve was found for patients with parasitic/fungal infections, followed by those with viral infections, whereas the worst survival curves were for those with bacterial infections and multiple microbiological infections (of whom 71% also had bacterial infections in association with other microorganisms).…”

Section: Discussionmentioning

confidence: 99%

Prognostic Factors of Death in 151 Adults With Hemophagocytic Syndrome: Etiopathogenically Driven Analysis

Brito-Zerón¹,

Kostov

Moral-Moral

et al. 2018

Mayo Clinic Proceedings: Innovations, Quality & Outcomes

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ObjectiveTo characterize the etiologies and clinical features at diagnosis of patients with hemophagocytic lymphohistiocytosis (HLH) and correlate these baseline features with survival using an etiopathogenically guided multivariable model.Patients and MethodsThe Spanish Group of Autoimmune Diseases HLH Study Group, formed in 2013, is aimed at collecting adult patients with HLH diagnosed in internal medicine departments between January 3, 2013, and October 28, 2017.ResultsThe cohort consisted of 151 patients (91 men; mean age, 51.4 years). After a mean follow-up of 17 months (range, 1-142 months), 80 patients died. Time-to-event analyses for death identified a worse survival curve for patients with neoplasia (P<.001), mixed microbiological infections (P=.02), and more than 1 infection (P=.01) and glucocorticoid monotherapy (P=.02). According to univariate analyses, platelets of less than 100,000/mm3 (hazard ratio [HR], 3.39; 95% CI, 1.37-8.40), leukopenia (HR, 1.81; 95% CI, 1.01-3.23), severe hyponatremia (HR, 1.61; 95% CI, 1.02-2.54), disseminated intravascular coagulation (HR, 1.87; 95% CI, 1.05-3.34), bacterial infection (HR, 1.99; 95% CI, 1.09-3.63), mixed microbiological infections (HR, 3.42; 95% CI, 1.38-8.46), and 2 or more infectious triggers (HR, 2.95; 95% CI, 1.43-6.08) were significantly associated with death. In contrast, peripheral adenopathies (HR, 0.63; 95% CI, 0.40-0.98) and the immunosuppressive drug/intravenous immunoglobulin/biological therapies (HR, 0.44; 95% CI, 0.20-0.96) were protective against all-cause mortality. Multivariable Cox proportional hazards regression analysis identified 2 or more infectious triggers (HR, 3.14; 95% CI, 1.28-7.68) as the only variable independently associated with death.ConclusionThe mortality rate of adult patients diagnosed with HLH exceeds 50%. Infection with more than 1 microbiological agent was the only independent variable associated with mortality irrespective of the underlying disease, epidemiological profile, clinical presentation, and therapeutic management.

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“…HLH can simulate infections/sepsis, hepatitis, multiple organ dysfunction syndrome, encephalitis, autoimmune lymphoproliferative syndrome, thrombotic thrombocytopenic purpura, or fever of unknown origin. Infection has been shown in a study to be the major trigger of HLH in adults treated with biological therapies [9]. …”

Section: Discussionmentioning

confidence: 99%

Histoplasmosis-Induced Hemophagocytic Lymphohistiocytosis in an Adult Patient: A Case Report and Review of the Literature

Untanu

Akbar

Graziano

et al. 2016

Case Reports in Infectious Diseases

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Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threating immune dysregulation syndrome characterized by persistent activation of the mononuclear phagocytic system leading to uncontrolled systemic hyperinflammatory response. The proliferation and activation of histiocytes and lymphocytes lead to production of large amounts of cytokines, also called cytokine storm. Hematopoietic and lymphoid tissues are directly involved while other organs are damaged by circulating cytokines. Primary HLH is attributed to genetic defects of the immune system and secondary HLH is usually seen in adults secondary to malignancy, infection, or autoimmune diseases. Zoonotic diseases including fungal infections are an important cause of HLH. Secondary HLH can delay the recognition of the underlying zoonoses. We report the case of a 61-year-old female with history of rheumatoid arthritis with histoplasmosis associated hemophagocytic lymphohistiocytosis.

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Infection is the major trigger of hemophagocytic syndrome in adult patients treated with biological therapies

Cited by 55 publications

References 44 publications

Impact of Epstein‐Barr virus serological status on clinical outcomes in adult patients with inflammatory bowel disease

Impact of Epstein‐Barr virus serological status on clinical outcomes in adult patients with inflammatory bowel disease

Prognostic Factors of Death in 151 Adults With Hemophagocytic Syndrome: Etiopathogenically Driven Analysis

Histoplasmosis-Induced Hemophagocytic Lymphohistiocytosis in an Adult Patient: A Case Report and Review of the Literature

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