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      Severe Pneumocystis jirovecii pneumonia in an idiopathic CD4 + lymphocytopenia patient: case report and review of the literature

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          When diagnosing Pneumocystis jirovecii pneumonia (PJP), the clinical suspicion must be confirmed by laboratory tests. PJP is rarely described in patients with idiopathic CD4 + lymphocytopenia (ICL), a rare T-cell deficiency of unknown origin with persistently low levels of CD4 + T-cells (<300 µl −1 or <20 % of total lymphocytes) but repeated negative human immunodeficiency virus (HIV) tests. We retrospectively analysed a case of an ICL patient with severe PJP associated with multiple opportunistic infections (OIs). We also reviewed the literature since 1986.

          Case presentation:

          A laboratory-confirmed case of PJP associated with invasive candidiasis and cytomegalovirus infection was reported in an ICL patient. Despite early treatment, the patient died of respiratory failure under polymicrobial pneumonia. According to the literature, the mortality rate of ICL patients is 10.4 % (33/316). In ICL patients, the risk of OI is 83.2 % (263/316), with viral infections being the most prevalent (58.2 %, 184/316), followed by fungal infections (52.2 %, 165/316) and mycobacterial infections (15.5 %, 49/316). Dysimmunity is reported in 15.5 % (49/316) of ICL patients. Among the fungal infections, cryptococcal infections are the most prevalent (24.1 %, 76/316), followed by candidiasis (15.5 %, 49/316) and PJP (7.9 %, 25/316).


          The high risk of OIs underlines the importance of more vigorous preventative actions in hospitals. The response to therapy and the detection of early relapse of PJP may be monitored by several laboratory tests including quantitative PCR. It is essential to treat the ICL and to follow the guidelines concerning therapy and prophylaxis of OIs as given to HIV patients.

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          Most cited references 42

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          The PATH (Prospective Antifungal Therapy) Alliance® registry and invasive fungal infections: update 2012.

          The Prospective Antifungal Therapy Alliance (PATH Alliance®) performed prospective surveillance of invasive fungal infections (IFIs) among patients hospitalized at 25 medical centers in North America between 2004 and 2008, collecting information on the epidemiology, diagnosis, treatment, and mortality rates of IFIs. In total, 7526 IFIs were identified in 6845 patients. Candida spp. (73.4%) were the most common pathogens, followed by Aspergillus spp. (13.3%), and other yeasts (6.2%). Culture was the most frequently used diagnostic test in the majority of IFI categories. Most patients with invasive candidiasis were treated with fluconazole (48.3%) and the echinocandins (34.0%), while voriconazole (45.5%) was the main antifungal agent for invasive aspergillosis. The 12-week survival rate ranged from 37.5% for hematopoietic stem cell transplant recipients to ~75.0% for those with HIV/AIDS. In summary, the findings of the PATH Alliance® registry provide a better understanding of the epidemiology of a vast variety and large numbers of IFIs. Copyright © 2012 Elsevier Inc. All rights reserved.
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            Real-time PCR assay-based strategy for differentiation between active Pneumocystis jirovecii pneumonia and colonization in immunocompromised patients.

            Diagnosis of pneumocystosis usually relies on microscopic demonstration of Pneumocystis jirovecii in respiratory samples. Conventional PCR can detect low levels of P. jirovecii DNA but cannot differentiate active pneumonia from colonization. In this study, we used a new real-time quantitative PCR (qPCR) assay to identify and discriminate these entities. One hundred and sixty-three bronchoalveolar lavage fluids and 115 induced sputa were prospectively obtained from 238 consecutive immunocompromised patients presenting signs of pneumonia. Each patient was classified as having a high or a low probability of P. jirovecii pneumonia according to clinical and radiological presentation. Samples were processed by microscopy and by a qPCR assay amplifying the P. jirovecii mitochondrial large-subunit rRNA gene; qPCR results were expressed as trophic form equivalents (TFEq)/mL by reference to a standard curve obtained from numbered suspensions of trophic forms. From 21 samples obtained from 16 patients with a high probability of P. jirovecii pneumonia, 21 were positive by qPCR whereas only 16 were positive by microscopy. Fungal load ranged from 134 to 1.73 × 10(6)  TFEq/mL. Among 257 specimens sampled from 222 patients with a low probability of P. jirovecii pneumonia, 222 were negative by both techniques but 35 were positive by qPCR (0.1-1840 TFEq/mL), suggesting P. jirovecii colonization. Two cut-off values of 120 and 1900 TFEq/mL were proposed to discriminate active pneumonia from colonization, with a grey zone between them. In conclusion, this qPCR assay discriminates active pneumonia from colonization. This is particularly relevant for patient management, especially in non-human immunodeficiency virus (HIV)-infected immunocompromised patients, who often present low-burden P. jirovecii infections that are not diagnosed microscopically. © 2011 The Authors. Clinical Microbiology and Infection © 2011 European Society of Clinical Microbiology and Infectious Diseases.
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              Clinical manifestations of Cryptococcus gattii infection: determinants of neurological sequelae and death.

              Longer-term morbidity and outcomes of Cryptococcus gattii infection are not described. We analyzed clinical, microbiological, and outcome data in Australian patients followed for 12 months, to identify prognostic determinants. Culture-confirmed C. gattii cases from 2000 to 2007 were retrospectively evaluated. Clinical, microbiological, radiological, and outcome data were recorded at diagnosis and at 6 weeks, 6 months, and 12 months. Clinical and laboratory variables associated with mortality and with death and/or neurological sequelae were determined. Annual C. gattii infection incidence was 0.61 per 10(6) population. Sixty-two of 86 (72%) patients had no immunocompromise; 6 of 24 immunocompromised hosts had idiopathic CD4 lymphopenia, and 1 had human immunodeficiency virus/AIDS. Clinical and microbiological characteristics of infection were similar in immunocompromised and healthy hosts. Isolated lung, combined lung and central nervous system (CNS), and CNS only disease was reported in 12%, 51% and 34% of the cases, respectively. Complications in CNS disease included raised intracranial pressure (42%), hydrocephalus (30%), neurological deficits (27%; 6% developed during therapy) and immune reconstitutionlike syndrome (11%). Geometric mean serum cryptococcal antigen (CRAG) titers in CNS disease were 563.9 (vs 149.3 in isolated lung infection). Patient immunocompromise was associated with increased mortality risk. An initial cerebrospinal fluid CRAG titer of ≥256 predicted death and/or neurological sequelae (P = .05). Neurological C. gattii disease predominates in the Australian endemic setting. Lumbar puncture and cerebral imaging, especially if serum CRAG titers are ≥512, are essential. Long-term follow up is required to detect late neurological complications. Immune system evaluation is important because host immunocompromise is associated with reduced survival.

                Author and article information

                JMM Case Rep
                JMM Case Rep
                JMM Case Reports
                Microbiology Society
                December 2014
                1 December 2014
                : 1
                : 4
                [ 1]Laboratory of Parasitology – Mycology, Grenoble University Hospital, CS 10217, F-38043 Grenoble, France
                [ 2]Laboratory of Parasitology – Mycology, University Hospital of Saint Etienne, Saint Etienne, Av Albert Raimond, F-42055 Saint Etienne, France
                [ 3]Laboratory of Virology, Grenoble University Hospital, CS 10217, F-38043 Grenoble, France
                [ 4]Intensive Care Unit, Grenoble University Hospital, CS 10217, F-38043 Grenoble, France
                [ 5]Internal Medicine, Grenoble University Hospital, CS 10217, F-38043 Grenoble, France
                [ 6]Université de Grenoble Alpes, Grenoble, France
                [ 7]Jean Monnet University, Saint Etienne, France
                Author notes
                Bernabé F. F. Chumpitazi BChumpitazi@
                © 2014 The Authors

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License (

                Case Review


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