IVIG Treatment of Adenovirus Infection-Associated Macrophage Activation Syndrome in a Two-Year-Old Boy: Case Report and Review of the Literature

Abstract

A 2-year-old boy with severe multiorgan disease (i.e., otitis, enteritis, bilateral pneumonia, encephalopathy, myocarditis, rash) was diagnosed with adenovirus-associated macrophage activation syndrome according to clinical and laboratory parameters (fever, hepatosplenomegaly, bicytopenia, hyperferritinemia, hypertriglyceridemia). He had no unusual history of earlier infections or a family history of hemophagocytic syndrome or immune defects. Intravenous immunoglobulin was administered to prevent exacerbation of the suspected incipient hemophagocytic syndrome. Clarithromycin, a macrolide with immunomodulatory effect, was included in the antibiotic regimen of the progressive pneumonia, followed by rapid amelioration and remission of clinical and laboratory findings. Causal links between treatment and clinical improvement are discussed and a brief review of the recent literature is included.

Weathering the Storm: Improving Therapeutic Interventions for Cytokine Storm Syndromes by Targeting Disease Pathogenesis

“…Therefore, the cornerstone of therapy for MAS directly targets the immune mechanisms that drive the hyperinflammatory response in MAS. Intriguingly, intravenous immunoglobulin is the most commonly used therapy for MAS other than corticosteroids and cyclosporine (61), although evidence for its benefit is limited to observational studies in a heterogeneous population of cytokine storm syndrome patients not limited to MAS (65, 66) (Level of Evidence 4). Anakinra may be of benefit to patients with refractory MAS (60) (Level of evidence 4), although other biologic therapies including canakinumab and tocilizumab have not demonstrated benefit in MAS, as detailed above (57–59).…”

Severe Adenovirus Infection Associated with Hemophagocytic Lymphohistiocytosis

“…However, in immunocompromised patients, adenovirus can cause fulminant or disseminated disease such as colitis, pneumonitis, pancreatitis, nephritis, encephalitis, and hemophagocytic lymphohistiocytosis (HLH) [1,2]. Herein, we report the clinical course and the treatment of an infant who had no history of immune defects, familial HLH, or malignant disease and suffered from adenoviral pneumonia with progressive clinical deterioration due to onset of virus-associated HLH.…”

“…There have been a limited number of case reports describing adenovirus pneumonia complicated with HLH [2,4,5,6]. In these reports, adenoviral pneumonia with HLH was successfully treated with IVIG and clarithromycin, dexamethasone and cyclosporine, or pulse methylprednisolone [2,4,6].…”

“…There have been a limited number of case reports describing adenovirus pneumonia complicated with HLH [2,4,5,6]. In these reports, adenoviral pneumonia with HLH was successfully treated with IVIG and clarithromycin, dexamethasone and cyclosporine, or pulse methylprednisolone [2,4,6]. Antiviral treatment with cidofovir or ribavirin in adenovirus infections is being increasingly used, especially in immunocompromised patients; however, the efficacy of these drugs is not established [7,8].…”

Epstein-Barr virus-associated hemophagocytic syndrome in a patient with lupus nephritis

“…The role of IVIG in the treatment HPS is unclear. Remission after IVIG therapy has been reported in adults and children with underlying immune dysfunction [9,10]. Since IVIG therapy is immunoregulatory but not immunosuppressive or myelotoxic, it seems to be reasonable in both types of SLE-associated HPS.…”