Adjuvant Corticosteroid Therapy in 2 Children With Hepatosplenic Candidiasis-related IRIS

Abstract

First described in HIV-infected patients who recently initiated highly active antiretroviral therapy, the immune reconstitution inflammatory syndrome (IRIS) is best characterized as a collection of inflammatory disorders triggered by rapid resolution of immunosuppression. Treatment of IRIS is a clinical challenge due to the variety of clinical presentations and the presence of multiple pathogens capable of causing the syndrome. Hepatosplenic candidiasis, an uncommon form of invasive Candida species infection, was recently suggested to belong to the spectrum of fungus-related IRIS. We report 2 cases of probable hepatosplenic candidiasis according to the guidelines of the European Organization for Research and Treatment of Cancer and the Mycosis Study Group, occurring in pediatric patients with acute leukemia during rapid neutrophil recovery after cytotoxic chemotherapy. In both cases, abdominal computed tomography scan revealed multiple hepatic micronodules, and liver biopsy showed nonspecific granulomatous lesions. Hepatosplenic candidiasis symptoms (fever, nausea and vomiting, abdominal pain) resolved within 2 days after adjunction of corticosteroid therapy to antifungal treatment. Inflammatory markers and related radiologic abnormalities decreased or disappeared within 1 month. Recovery of neutrophil count in a context of hepatosplenic candidiasis may result in a heightened inflammatory response. Corticosteroid therapy in this setting is associated with prompt resolution of the symptoms.

Adjuvant corticosteroid therapy in hepatosplenic candidiasis-related IRIS

“…Delay in the treatment of leukemia may affect the course and survival of these patients. As recently reported, addition of corticosteroid therapy to antifungal therapy in patients with HSC related IRIS has found to be associated with disappearance of clinical symptoms, radiologic abnormalities and inflammatory markers 6,14. It was supposed that corticosteroids might adjust the paradoxical immune response by modulating both mononuclear phagocyte function and T-cell activation 6.…”

Candida-Related Immune Response Inflammatory Syndrome Treated with Adjuvant Corticosteroids and Review of the Pediatric Literature

“…The most commonly administered antifungal agent was amphotericin B. Details of steroid therapy and the outcomes are presented in Table 1 [3,7,8,9,10,11]. Increased susceptibility to infection might be a drawback for prolonged corticotherapy.…”

New insights into hepatosplenic candidosis, a manifestation of chronic disseminated candidosis

“…An interesting aspect of the disease is the rapid clinical improvement seen with the adjunct use of corticosteroids compared with the slow and sometimes partial efficiency of antifungal treatment alone 52 . Moreover, a recent publication showed resolution of HSC symptoms as early as two days of corticosteroids in combination with antifungal treatment in two leukaemic children 53 . The benefit of corticosteroids is known in HIV patients on antiretroviral therapy who had cryptococcosis or histoplasmosis complicated by an immune reconstitution inflammatory syndrome (IRIS) 54–57 .…”

“…52 Moreover, a recent publication showed resolution of HSC symptoms as early as two days of corticosteroids in combination with antifungal treatment in two leukaemic children. 53 The benefit of corticosteroids is known in HIV patients on antiretroviral therapy who had cryptococcosis or histoplasmosis complicated by an immune reconstitution inflammatory syndrome (IRIS). [54][55][56][57] It has therefore been postulated that the pathophysiology of HSC is somewhat similar to IRIS.…”