Late Onset Combined Immunodeficiency Presenting with Recurrent Pneumocystis jiroveci Pneumonia

Late onset combined immunodeficiency (LOCID) is a recently described variant of common variable immunodeficiency (CVID), involving adult patients presenting with opportunistic infections and/or low CD4+ lymphocyte counts. A 36-year-old male with unremarkable past medical history presented with fever...

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Main Authors: Ilias Papakonstantinou, Ioannis G. Baraboutis, Lazaros Karnesis
Format: Article
Language:English
Published: Wiley 2014-01-01
Series:Case Reports in Medicine
Online Access:http://dx.doi.org/10.1155/2014/801805
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author Ilias Papakonstantinou
Ioannis G. Baraboutis
Lazaros Karnesis
author_facet Ilias Papakonstantinou
Ioannis G. Baraboutis
Lazaros Karnesis
author_sort Ilias Papakonstantinou
collection DOAJ
description Late onset combined immunodeficiency (LOCID) is a recently described variant of common variable immunodeficiency (CVID), involving adult patients presenting with opportunistic infections and/or low CD4+ lymphocyte counts. A 36-year-old male with unremarkable past medical history presented with fever, respiratory failure, and lymphocytopenia. He was found to have Pneumocystis jiroveci pneumonia (PJP), subsequently complicated by recurrent hospital-acquired Pseudomonas aeruginosa pneumonia and immune reconstitution phenomena, attributed to restoration of immunoglobulin levels. Clinicians should be aware of LOCID, which could be confused with HIV infection/AIDS or idiopathic CD4 lymphocytopenia. In the English bibliography there is only one case report, where PJP was the initial presentation of CVID (that case would probably be classified as LOCID). Phenomena of immune reconstitution are described in various settings, including primary immunodeficiency, manifesting as temporary clinical and radiologic deterioration and leading to misperceptions of therapeutic failure and/or presence of alternative/additional diagnoses.
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institution Kabale University
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spelling doaj-art-06be021df8db4c93bdd28e025f636dcd2025-02-03T00:59:47ZengWileyCase Reports in Medicine1687-96271687-96352014-01-01201410.1155/2014/801805801805Late Onset Combined Immunodeficiency Presenting with Recurrent Pneumocystis jiroveci PneumoniaIlias Papakonstantinou0Ioannis G. Baraboutis1Lazaros Karnesis21st Department of Internal Medicine, 401 General Military Hospital of Athens, Mesogeion Avenue 138, 11525 Athens, GreeceInfectious Diseases & HIV Division of the 5th Department of Internal Medicine, “Evangelismos” General Hospital, 45-47 Ipsilantou Street, 10676 Athens, Greece1st Department of Internal Medicine, 401 General Military Hospital of Athens, Mesogeion Avenue 138, 11525 Athens, GreeceLate onset combined immunodeficiency (LOCID) is a recently described variant of common variable immunodeficiency (CVID), involving adult patients presenting with opportunistic infections and/or low CD4+ lymphocyte counts. A 36-year-old male with unremarkable past medical history presented with fever, respiratory failure, and lymphocytopenia. He was found to have Pneumocystis jiroveci pneumonia (PJP), subsequently complicated by recurrent hospital-acquired Pseudomonas aeruginosa pneumonia and immune reconstitution phenomena, attributed to restoration of immunoglobulin levels. Clinicians should be aware of LOCID, which could be confused with HIV infection/AIDS or idiopathic CD4 lymphocytopenia. In the English bibliography there is only one case report, where PJP was the initial presentation of CVID (that case would probably be classified as LOCID). Phenomena of immune reconstitution are described in various settings, including primary immunodeficiency, manifesting as temporary clinical and radiologic deterioration and leading to misperceptions of therapeutic failure and/or presence of alternative/additional diagnoses.http://dx.doi.org/10.1155/2014/801805
spellingShingle Ilias Papakonstantinou
Ioannis G. Baraboutis
Lazaros Karnesis
Late Onset Combined Immunodeficiency Presenting with Recurrent Pneumocystis jiroveci Pneumonia
Case Reports in Medicine
title Late Onset Combined Immunodeficiency Presenting with Recurrent Pneumocystis jiroveci Pneumonia
title_full Late Onset Combined Immunodeficiency Presenting with Recurrent Pneumocystis jiroveci Pneumonia
title_fullStr Late Onset Combined Immunodeficiency Presenting with Recurrent Pneumocystis jiroveci Pneumonia
title_full_unstemmed Late Onset Combined Immunodeficiency Presenting with Recurrent Pneumocystis jiroveci Pneumonia
title_short Late Onset Combined Immunodeficiency Presenting with Recurrent Pneumocystis jiroveci Pneumonia
title_sort late onset combined immunodeficiency presenting with recurrent pneumocystis jiroveci pneumonia
url http://dx.doi.org/10.1155/2014/801805
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AT ioannisgbaraboutis lateonsetcombinedimmunodeficiencypresentingwithrecurrentpneumocystisjirovecipneumonia
AT lazaroskarnesis lateonsetcombinedimmunodeficiencypresentingwithrecurrentpneumocystisjirovecipneumonia