A case study of disseminated histoplasmosis linked to common variable immunodeficiency

Rachid, Acir; Rezende, Lucila Stange; Moura, Suzelle Freitas de; Loffy, Paulo C.; Magalhães, Francisco Luiz Gomide M.

doi:10.1590/s1413-86702003000400007

Cited by 10 publications

(8 citation statements)

References 11 publications

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“…CVID, however, comprises a heterogeneous group of disorders, which often include abnormalities in T-cell phenotype and function, secretion of cytokines, or T-cell receptor signalling events [1,64,76]. A number of case reports of IFIs in CVID patients have been published, including histoplasmosis (meningitis and disseminated infection), aspergillosis (lung infection and liver abscess) and disseminated infection caused by Penicillium marneffei [77][78][79][80][81][82].…”

Section: Humoral Immunodeficienciesmentioning

confidence: 99%

Invasive fungal infections in congenital immunodeficiencies

Antachopoulos

2010

Clinical Microbiology and Infection

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Both acquired and congenital immunodeficiencies may be associated with increased susceptibility to invasive fungal infections (IFIs), depending on the type of immune deficit. IFIs frequently occur in patients with phagocytic and cellular immune defects, but are rarely observed in those with humoral or complement deficits. Among congenital immune disorders, chronic granulomatous disease and hyper-IgE syndrome are most frequently associated with IFIs; variable susceptibility to fungal pathogens is also seen in patients with severe combined immunodeficiency, X-linked hyper-IgM syndrome, Wiskott-Aldrich syndrome, DiGeorge syndrome, common variable immunodeficiency, defects in the interferon-γ-interleukin-12 axis, and myeloperoxidase deficiency. Aspergillus, Candida, Cryptococcus, Histoplasma and other fungal genera are variably implicated in causing invasive infections in these patients. Prompt diagnosis of IFIs in this patient population requires a high degree of suspicion, together with a knowledge of their clinical presentation and the limitations of diagnostic modalities. Apart from administration of appropriate antifungal agents, successful management often requires the addition of surgical intervention. Adjunctive immunotherapy may be considered, although this has not been systematically studied. Prophylactic interferon-γ and itraconazole administration have been shown to reduce the risk of IFIs in patients with chronic granulomatous disease; however, the possibility of infections with azole-resistant organisms following long-term itraconazole prophylaxis should not be overlooked.

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Section: Humoral Immunodeficienciesmentioning

confidence: 99%

Invasive fungal infections in congenital immunodeficiencies

Antachopoulos

2010

Clinical Microbiology and Infection

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“…Oropharyngeal candidiasis has been reported among CVID patients and an increased susceptibility to ex vivo nail infection with C. albicans and Trichophyton mentagrophytes has been demonstrated [95,130]. Histoplasma capsulatum has been implicated in disseminated infection (with manifestations from the gastrointestinal, lower respiratory and central nervous system) [73,131] and isolated meningitis [34]. Invasive aspergillosis has been reported in two cases, manifesting as pulmonary infection or hepatic abscess [162,164].…”

Section: Common Variable Immunodeficiency (Cvid)mentioning

confidence: 99%

Fungal infections in primary immunodeficiencies

2007

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Patients with phagocytic, cellular, combined and other primary immunodeficiencies exhibit immune deficits that confer increased susceptibility to fungal infections. A number of yeasts and moulds, most commonly Candida and Aspergillus but also Cryptococcus, Histoplasma, Paecilomyces, Scedosporium, Trichosporon, Penicillium and other, rarely isolated, fungal organisms, have been variably implicated in causing disease in patients with chronic granulomatous disease, severe combined immunodeficiency, chronic mucocutaneous candidiasis, hyper-IgE syndrome, myeloperoxidase deficiency, leukocyte adhesion deficiency, defects in the interferon-gamma/interleukin-12 axis, DiGeorge syndrome, X-linked hyper-IgM syndrome, Wiskott-Aldrich syndrome and common variable immunodeficiency. Differences in the spectrum of fungal pathogens as well as in the incidence and clinical presentation of the infections may be observed among patients, depending upon different immune disorders. Fungal infections in these individuals may occasionally be the presenting clinical manifestation of a primary immunodeficiency and can cause significant morbidity and potentially fatal outcome if misdiagnosed or mistreated. A high degree of suspicion is needed and establishment of diagnosis should actively be pursued using appropriate imaging, mycological and histological studies. A number of antifungal agents introduced over the last fifteen years, such as the lipid formulations of amphotericin B, the second-generation triazoles, and the echinocandins, increase the options for medical management of these infections. Surgery may also be needed in some cases, while the role of adjunctive immunotherapy has not been systematically evaluated. The low incidence of primary immunodeficiencies in the general population complicates single-center prospective or retrospective clinical studies aiming to address diagnostic or therapeutic issues pertaining to fungal infections in these patients.

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“…Splenomegaly in CVID has been attributed to the lower number of T regulatory cells resulting in chronic and ineffective/nonselective activation of T cells with inverse CD4/CD8 ratios and polyclonal expansion of large granular lymphocytes [18] , [19] , [20] . This abnormal T cell activation has been linked to the risk of disseminated histoplasmosis in this population [3] . In our case, as splenomegaly improved with antifungal therapy, it is likely a manifestation of disseminated histoplasmosis as a result of such abnormal T cell regulation in CVID.…”

Section: Discussionmentioning

confidence: 95%

“…Sporadic and inherited forms of Common variable immunodeficiency (CVID), is classically described as hypogammaglobulinemia from B cell defects predisposing to recurrent bacterial sino-pulmonary infections. Abnormalities in T cells and antigen presenting cells have also been rarely reported [2] , [3] , [4] . Literature on disseminated histoplasmosis in patients with CVID is sparse [3] , [5] , [6] , [7] , [8] , [9] .…”

Section: Introductionmentioning

confidence: 99%