Chronic Disseminated Candidiasis Complicated by Immune Reconstitution Inflammatory Syndrome in Child with Acute Lymphoblastic Leukemia

Abstract

Hepatosplenic candidiasis also known as chronic disseminated candidiasis is a rare manifestation of invasive fungal infection typically observed in patients with acute leukemia in prolonged, deep neutropenia. Immune reconstitution inflammatory syndrome (IRIS) is an inflammatory disorder triggered by rapid resolution of neutropenia. Diagnosis and treatment of IRIS are still challenging due to a variety of clinical symptoms, lack of certain diagnostic criteria, and no standards of treatment. The diagnosis of IRIS is even more difficult in patients with hematological malignancies complicated by “probable” invasive fungal infection, when fungal pathogen is still uncertain. We report a case of probable hepatic candidiasis in 4-year-old boy with acute lymphoblastic leukemia. Despite proper antifungal therapy, there was no clinical and radiological improvement, so diagnosis of Candida-related IRIS was made and corticosteroid therapy was added to antifungal treatment achieving prompt resolution of infection symptoms.

Subcutaneous Microabscesses and Myositis as Part of Immune Reconstitution Inflammatory Syndrome due to Chronic Disseminated Candidiasis in a Child With Acute Lymphoblastic Leukemia

“…Although prednisolone is the most common type of steroid used, there are no trials or recommendations on its dosage and duration. Like in our patient, the majority of the reported cases have commenced prednisolone at 1 to 2 mg/kg and tapered over time, with the total duration ranging anywhere between 6 days and 8 months based on the clinical discretion of treating physicians 8–12. Our patient’s CRP reduced within a week but has had a persistent high neutrophil count.…”

Chronic Disseminated Candidiasis in Children and the Role of Corticosteroids Therapy

“…Diagnosis and management of CDC-related IRIS is always challenging especially in probable and possible cases. 2 CDC-related IRIS should be considered in children with persistent fever, exaggerated inflammatory response and persistent symptoms on antifungal therapy once other causes were excluded. 4,10 Response to corticosteroids with CDC induced IRIS is similar to other types of IRIS that are commonly seen with human immunodeficiency virus (HIV) patients.…”

“…2,4 Although this condition has been described for more than 50 years, its diagnosis and management are not well established yet. 2 CDC can be complicated by immune reconstitution inflammatory syndrome (IRIS) upon neutrophil recovery. 2,4 Management of CDC-related IRIS requires prolonged course of targeted antifungal therapy and short courses of corticosteroids in some selected patients.…”

“…2 CDC can be complicated by immune reconstitution inflammatory syndrome (IRIS) upon neutrophil recovery. 2,4 Management of CDC-related IRIS requires prolonged course of targeted antifungal therapy and short courses of corticosteroids in some selected patients. 2,4,5 There are limited data on the management of CDC in children and the role of corticosteroid for CDC-related IRIS in this population.…”

Rapid clinical response to adjuvant corticosteroids in chronic disseminated candidiasis complicated by granulomas and persistent fever in acute leukemia patients

“…The use of corticosteroids in the treatment of acute IFIs is controversial, appears counterintuitive and they are not recommended in treatment guidelines [6,7]. Whilst numbers reported in the literature are low, corticosteroids appear to be critical to clinical improvement in IFI patients who have developed IRIS (see table 1 [5,[8][9][10][11][12][13][14]). Here, we The understanding of the pathophysiology of the IRIS associated with IFIs is incomplete.…”