Disseminated Histoplasmosis and Associated Allergic Bronchopulmonary Aspergillosis in a Patient with Hyper-IgE Immunodeficiency

A. M. Arenas-Rojas1, D. T. Gómez-Alzate1, S. Y. Moreno-Lozano2, P. E. Sarmiento-Wilches3;

Author address: 

1Clínica Materno-Infantil San Luis, Bucaramanga, Colombia, 2Pediatric Pulmonology, Clínica Materno-Infantil San Luis, Bucaramanga, Colombia, 3Pediatric Infectious Diseases, Clínica Materno-Infantil San Luis, Bucaramanga, Colombia.

Abstract: 

Introduction: Histoplasmosis is a fungal infection caused by endemic dimorphic fungus Histoplasma capsulatum. It is the most common cause of invasive pulmonary disease by fungi in humans. Severe clinical presentations such as disseminated histoplasmosis can develop in immunocompromised children. Diagnostic approach can be complex due to low clinical suspicion, and in the case of Colombia, difficult access to required diagnostic tests, which causes delay in treatment and higher rates of morbidity and mortality.
Case report: A 17-year-old male, presented with fever, non-productive cough and sore throat for 7 days. Patient had a history of skin lesions since 2 months of age, xeroderma, eczema, recurrent skin abscesses, oral candidiasis, malocclusion caused by delayed exfoliation of deciduous teeth, and approximately 1 or 2 episodes of pneumonia per year. He presented with rapid deterioration and was admitted to the ICU due to risk of respiratory failure. On physical examination, broad nasal bridge, prognathism and oral candidiasis were present. Pulmonary auscultation revealed rhonchi and crepitus on both lung fields. Initial chest Computed Tomography (CT) showed involvement of the parenchyma, diffusereticulomicronodular pattern with bilateral upper and lower lobe involvement, alveolar occupation pattern with distribution in patches that tended to coalesce toward the pulmonary apices, and right basal consolidation. Laboratory tests showed absolute eosinophil count of 530/mm3, lymphocyte T CD3 407cel/uL, CD4 197cel/uL and CD8 193cel/uL, total IgE 4863UI/mL, and Aspergillus fumigatus-specific IgE class 1 levels 0.46kU/L. Broncho-alveolar lavage was negative for Mycobacterium spp and fungi. Giemsa and Grocott stained bone marrow aspirate reported small, round, grouped intracytoplasmic structures suggestive of Histoplasma. We concluded the diagnosis of hyper-IgE syndrome with disseminated histoplasmosis and associated Allergic Broncho-Pulmonary Aspergillosis (ABPA). Treatment was started with liposomal amphotericin B, but due to acute nephrotoxicity it was changed to itraconazole after completing the minimum 2 weeks recommended. Chest CT after 25 days of treatment revealed that the tree-in-bud pattern had disappeared, there was no alveolar occupation and there was only a residual image of right basal consolidation.
Discussion: Hiper-IgE syndrome is an uncommon pathology, there are few reported cases worldwide. Its association with aspergillosis is rare, in this case it was caused by a hypersensitivity reaction to Aspergillus antigens. The most frequent presentation in these patients is due to colonization of pneumatoceles forming aspergillomas. The importance of this case lies in the correlation of Hyper-IgE immunodeficiency with disseminated histoplasmosis and ABPA, which has not been described as a frequent association.

Tables: 

2018

abstract No: 

A5665 / P1112

Full conference title: 

The American Thoracic Society Conference 2018
    • ATS 2018