Clare Halfhide (United Kingdom)
Alder Hey Children's Hospital Paediatric Respiratory MedicineAuthor Of 1 Presentation
A NOT SO SIMPLE LYMPHADENITIS.
Abstract
Title of Case(s):
A Not So Simple Lymphadenitis.
Background:
Lymphadenitis caused by Candida species is an uncommon occurrence but has been observed in children with underlying immunodeficiency disorders such as chronic granulomatous disease (CGD).
Case Presentation Summary:
A 2 month-old, term male born to non-consanguineous parents, presented to hospital with acute respiratory distress and cervical lymphadenitis. His chest radiograph demonstrated left lung hyperinflation with significant right mediastinal shift. A chest CT scan identified a 2cm mediastinal lymph node causing complete occlusion of the left main bronchus while neck ultrasound scan showed multiple enlarged lymph nodes. A whole-body MRI scan did not detect any further abnormalities. Incisional biopsy of a cervical lymph node revealed multiple non-caseating granulomata with scanty yeast cells seen and Candida albicans was isolated from culture as well as 18S pan-fungal PCR. Mycobacterial and bacterial culture were negative. Bronchoalveolar lavage also detected C. albicans by PCR. Immune function investigations showed normal lymphocyte subsets, immunoglobulins and negative HIV serology, however his neutrophil oxidative burst test was abnormal confirming the diagnosis of CGD (<1% of neutrophils stimulated). Genetic testing identified a hemizygous CYBB variant of uncertain significance that has been implicated in X-linked CGD. He was commenced on steroid therapy and liposomal amphotericin with transition to oral posaconazole prior to transfer to a regional paediatric immunology centre where he received a haematopoietic-stem-cell-transplant from an unrelated-matched donor with good result.
Key Learning Points:
CGD is a rare inherited disorder of the oxidase pathway within neutrophils leading to the failure to generate microbicidal reactive oxidant anions and metabolites including hydrogen peroxide. This leads to recurrent life-threatening bacterial and fungal infections, with Staphylococcus aureus and Aspergillus being most common. Candida accounts for 6-14% of all invasive fungal infections in CGD and is the leading cause of fungal-related lymphadenitis, fungaemia and meningitis in this population. Candida lymphadenitis should prompt clinicians to investigate for CGD.