Pulmonary Cryptococcus infections as a manifestation of idiopathic CD4 lymphocytopenia: case report and literature review

Abstract

Background Idiopathic CD4 lymphocytopenia (ICL) is a rare clinical disease with relative CD4 deficiency in the absence of HIV infection. The pathogenicity of ICL is poorly understood with an unclear incidence rate in the general population. Sequelae of ICL includes AIDS-defining infections, which most commonly includes Cryptococcus neoformans. Typically, C. neoformans infections present with CNS involvement but rarely with extra-CNS manifestations. Here, we present a rare case of ICL with exclusively primary pulmonary cryptococcus and a review of the literature. Case presentation A 56-year-old female presented to our tertiary care hospital requiring a right hip open reduction intervention. The patient became febrile during admission, prompting a work-up that included a chest X-ray showing a peripheral pulmonary solitary nodule. Transthoracic biopsy revealed encapsulated yeast forms in keeping with C. neoformans. CD4 counts, repeated at least one month apart, were < 200 cells/mm3, with negative HIV testing. Flow cytometry and genetic testing were completed to elucidate the etiology of the immune deficiency, both of which were unremarkable. She was subsequently treated with 12 months of posaconazole with clinical resolution. Conclusions Our patient highlights a rare clinical disease, which a review of literature revealed only five cases in the literature with exclusive pulmonary Cryptococcus in ICL/ This case demonstrates the strong clinical acumen required to properly diagnose and ultimately manage the patient.

Clinical features of cryptococcosis in patients with different immune statuses: a multicenter study in Jiangsu Province–China

“…A Th1-Th17 cytokine profile is associated with increased phagocytic activity and inhibition of the growth of Cryptococcus [7]. Our previous study revealed that patients with CD4 T lymphocyte cell counts greater than 378/µL were more likely to be asymptomatic and had a lower mortality rate than those with lower CD4+ T lymphocyte counts [8,9]. Similarly, another study confirmed that patients with CD4+ T lymphocyte counts lower than 400/μL were prone to disseminated cryptococcosis [10].…”

Correlation Between CD4 Cell Count, HIV Viral Load, and Chest CT Findings of AIDS-associated Pulmonary Cryptococcosis

“…Only few studies on PC have focused on the correlation between CT findings and CD4 cell count or HIV viral load in immunocompromised patients, and most of these studies are case reports or have a small sample size (10,11). On the other hand, the majority of studies with a larger sample size have concentrated on the clinical and imaging characteristics of Cryptococcus in different immune states (3,12) or have simply compared the clinical and imaging features of immunocompetent and immunocompromised patients (13).…”

Progressive disseminated histoplasmosis in idiopathic CD4 lymphocytopenia an underdiagnosed combination - a case report

“…Regent et al found cryptococcal infection (26.7%) as the most common opportunistic infection followed by mycobacterial infection (17%) and histoplasmosis (3.1%) in ICL [14]. Subsequently, around 66 more cases were reported till 2020 in the literature [15]. Among them, a cases series of 40 patients showed that opportunistic infections were present in 62.5% of ICL cases (human papilloma virus, cryptococcus meningitis, pneumocystis pneumonia and mycobacterial tuberculosis were most common infections) [15].…”

“…Subsequently, around 66 more cases were reported till 2020 in the literature [15]. Among them, a cases series of 40 patients showed that opportunistic infections were present in 62.5% of ICL cases (human papilloma virus, cryptococcus meningitis, pneumocystis pneumonia and mycobacterial tuberculosis were most common infections) [15]. The association of histoplasmosis and ICL is rare and CD4 count was not done in all reported immunocompetent cases.…”