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,...

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Main Authors: Christina S. Thornton, Oscar Larios, Jennifer Grossman, Thomas P. Griener, Steven Vaughan
Format: Article
Language:English
Published: BMC 2019-10-01
Series:BMC Infectious Diseases
Subjects:
Online Access:http://link.springer.com/article/10.1186/s12879-019-4453-x
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author Christina S. Thornton
Oscar Larios
Jennifer Grossman
Thomas P. Griener
Steven Vaughan
author_facet Christina S. Thornton
Oscar Larios
Jennifer Grossman
Thomas P. Griener
Steven Vaughan
author_sort Christina S. Thornton
collection DOAJ
description 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.
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spelling doaj.art-416ca14b76e84dc2bd4edb4d203eb25a2022-12-22T00:59:57ZengBMCBMC Infectious Diseases1471-23342019-10-011911510.1186/s12879-019-4453-xPulmonary Cryptococcus infections as a manifestation of idiopathic CD4 lymphocytopenia: case report and literature reviewChristina S. Thornton0Oscar Larios1Jennifer Grossman2Thomas P. Griener3Steven Vaughan4Division of Respirology, Department of Medicine, University of CalgaryDivision of Infectious Diseases, Department of Medicine, University of CalgaryDivision of Hematology and Hematological Malignancies, Department of Medicine, University of CalgaryDepartment of Pathology & Laboratory Medicine, Cumming School of Medicine, Department of Medicine, University of CalgaryDivision of Infectious Diseases, Department of Medicine, University of CalgaryAbstract 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.http://link.springer.com/article/10.1186/s12879-019-4453-xPulmonary cryptococcusCD4 lymphocytopenia
spellingShingle Christina S. Thornton
Oscar Larios
Jennifer Grossman
Thomas P. Griener
Steven Vaughan
Pulmonary Cryptococcus infections as a manifestation of idiopathic CD4 lymphocytopenia: case report and literature review
BMC Infectious Diseases
Pulmonary cryptococcus
CD4 lymphocytopenia
title Pulmonary Cryptococcus infections as a manifestation of idiopathic CD4 lymphocytopenia: case report and literature review
title_full Pulmonary Cryptococcus infections as a manifestation of idiopathic CD4 lymphocytopenia: case report and literature review
title_fullStr Pulmonary Cryptococcus infections as a manifestation of idiopathic CD4 lymphocytopenia: case report and literature review
title_full_unstemmed Pulmonary Cryptococcus infections as a manifestation of idiopathic CD4 lymphocytopenia: case report and literature review
title_short Pulmonary Cryptococcus infections as a manifestation of idiopathic CD4 lymphocytopenia: case report and literature review
title_sort pulmonary cryptococcus infections as a manifestation of idiopathic cd4 lymphocytopenia case report and literature review
topic Pulmonary cryptococcus
CD4 lymphocytopenia
url http://link.springer.com/article/10.1186/s12879-019-4453-x
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