Cases reported "Pneumonia, Pneumocystis"

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1/11. Surfactant protein D and KL-6 as serologic indicators of pneumocystis carinii pneumonia in a child with acute lymphoblastic leukemia.

    pneumocystis carinii pneumonia (PCP) in immunocompromised patients is one of the important causes of morbidity and mortality. Serum levels of surfactant protein D (SP-D) and KL-6 are useful biomarkers for understanding some pulmonary inflammatory responses in patients with idiopathic pulmonary fibrosis (IPF). We present a child with PCP following bone marrow transplantation (BMT) for acute lymphoblastic leukemia (ALL), for whom both SP-D and KL-6 in sera were simultaneously measured through the clinical course of PCP. Both serum levels of SP-D and KL-6 were rapidly elevated reflecting some inflammatory processes in the lung that chest X-ray films and CT scans showed as ground-glass attenuations. He died due to respiratory failure 40 days after PCP developed. A combination of the assays for SP-D and KL-6 in sera may be helpful in predicting the treatment results for PCP.
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ranking = 1
keywords = idiopathic
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2/11. trimetrexate glucuronate associated with anti-Kaposi sarcoma effect.

    trimetrexate glucuronate, a dihydrofolate reductase inhibitor related to methotrexate, was developed by Parke-Davis as an alternative antineoplastic agent for tumors, especially sarcomas, that had developed resistance to methotrexate. This is a report on a patient with AIDS who developed pneumocystis carinii pneumonia, which was treated with trimethoprim sulfamethoxazole (Bactrim) with poor response, then with pentamidine with poor response, and finally with trimetrexate glucuronate (Neutrexin) and leucovorin rescue, with good response. The patient also suffered from cutaneous and visceral Kaposi's sarcoma (KS), which had been treated with high- dose HCG1 and well recognized chemotherapeutic protocols. Both HCG and chemotherapy resulted in tumor regression. The patient's KS flared, however, when he developed pneumocystis pneumonia. When trimetrexate glucuronate and leucovorin rescue were administered, his tumor burden decreased significantly, suggesting that trimetrexate glucuronate may have some activity against KS. The regression of KS in this anecdotal observation may be secondary to a delayed response from HCG and/or chemotherapy, or secondary to a spontaneous partial regression. Such regression may only be of the decreased edema around the KS lesions and not the neoplastic tissue itself. If other clinicians see this same phenomenon, however, it is possible that trimetrexate glucuronate may have an anti-KS effect. Such future clinical observations would warrant further testing at the basic science level.
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ranking = 2.9909531305635
keywords = cd
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3/11. The causes of death in patients with human immunodeficiency virus infection: a clinical and pathologic study with emphasis on the role of pulmonary diseases.

    The clinical records and autopsy data of 75 patients dying with AIDS were reviewed to determine the frequency of individual diseases diagnosed premortem and postmortem, the significance of pulmonary processes found in the lungs at autopsy, and the clinical and pathologic causes of death. cytomegalovirus (CMV) infection was identified histologically either premortem or postmortem in 81% of patients. The lungs and adrenal glands were infected most commonly. Only one-half of CMV infections were recognized premortem. Pneumocystis pneumonia and Kaposi sarcoma occurred in 68% and 59% of patients, respectively, but were not unsuspected premortem in any patient. Visceral involvement with Kaposi sarcoma, however, was frequently recognized only at autopsy. While disseminated M. avium-intracellulare infection was common (31% of patients), histologically documented pulmonary disease was uncommon (3% of patients). Cryptococcal infection, diagnosed in 10 patients, was confined to the central nervous system in only 1 patient. toxoplasma, in contrast, infected the brain of only 6 patients. All 75 patients had one or more disease processes identified in their lungs or pleurae at autopsy. These processes included opportunistic infections in 76% of patients, neoplasms in 37% (Kaposi sarcoma in 36% and lymphoma in 3%), and other processes in 60%. The most prevalent pathogen, CMV was found in pulmonary tissue from 44 patients and caused significant disease in 21 patients. Five patients died due to CMV pneumonia. pneumocystis carinii was found at autopsy in 24 patients. In spite of treatment, pneumocystis pneumonia was fatal in 11 patients. One patient died with concomitant CMV and pneumocystis pneumonia. Kaposi sarcoma, identified in the lungs of 23 patients, led to death in 5 patients via upper airway obstruction, hemorrhage, or parenchymal destruction. Other fatal pulmonary processes included bacterial pneumonia in 9 patients, idiopathic diffuse alveolar damage in 5, cryptococcosis in 2, and pulmonary hemorrhage in 1. Specific clinical criteria were used to determine the cause of death due to organ system failure. Fifty-one percent of patients died due to respiratory failure; 16% from neurologic disease; 17% from hypotension that was not caused by respiratory, neurologic, or cardiac disease; and 3% from cardiac dysfunction. Thirteen percent of deaths did not meet the clinical criteria defining these 4 categories. This clinical assessment was combined with autopsy data to identify specific diseases as causes of death.(ABSTRACT TRUNCATED AT 400 WORDS)
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ranking = 1
keywords = idiopathic
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4/11. Pulmonary complications of combination therapy with cyclophosphamide and prednisone.

    Oral cyclophosphamide and prednisone are standard treatment for some neoplasms and necrotizing systemic vasculitis and are advocated with increasing frequency for idiopathic interstitial lung disease. During a 15-month period, we observed four cases of acute respiratory failure from pneumocystis carinii pneumonia (PCP) in patients treated with oral cyclophosphamide and prednisone. One patient each had polyarteritis nodosa, Wegener's granulomatosis, bronchiolitis obliterans with organizing pneumonia, and chronic lymphocytic leukemia with red blood cell aplasia. hypoalbuminemia (serum albumin level less than 3.0 g/dl) and daily therapy were associated with increased risk for development of PCP (p less than 0.05). None of the patients had leukopenia (less than 3,500/cu mm) or neutropenia (less than 1,000/cumm) at diagnosis. All were negative for the human immunodeficiency virus. patients receiving oral cyclophosphamide and prednisone may be at higher or increasing risk for PCP. A high index of suspicion and aggressive evaluation for opportunistic infection are needed in these patients; consideration for trimethoprim-sulfamethoxazole prophylaxis and development of more quantitative measures of immunosuppression are needed.
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ranking = 1
keywords = idiopathic
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5/11. Pulmonary presentation of Kaposi's sarcoma in a homosexual patient.

    In the past two years there has been an unprecedented outbreak of Kaposi's sarcoma (KS) among male homosexuals with idiopathic acquired cellular immunodeficiency. A homosexual patient is presented in whom KS was first recognized in the lung. Pulmonary presentation of KS has not been previously reported in this group of patients. Therefore, KS must be considered in the differential diagnosis of pulmonary infiltrates in patients with acquired immunodeficiency, even in the absence of skin or lymph node involvement.
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ranking = 1
keywords = idiopathic
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6/11. Combined autoimmune cytopenias.

    BACKGROUND. Autoimmune neutropenia (AIN) can occur in association with autoimmune hemolytic anemia (AIHA) and/or immune thrombocytopenic purpura (ITP), although these associations have not been studied in detail. methods AND RESULTS. Twenty cases of AIN were found in a group of 55 adults with unexplained neutropenia over a five-year period. Eight subjects with AIN had an associated AIHA and/or ITP (AIN ITP, n = 2; AIN AIHA, n = 2; AIN ITP AIHA, n = 4). Thorough investigations identified no underlying disease in four patients, and none has appeared during follow-up. Of the other 4, one was found to have been suffering from systemic lupus erythematosus when the combined immunocytopenia was diagnosed, one patient from idiopathic myelofibrosis, one from a combined variable immunodeficiency and the other from disseminated tuberculosis. These last three conditions, while sometimes associated with autoimmune cytopenias, has not been previously reported together with combined immunocytopenias. All patients responded to immunosuppressors, although severe infectious complications occurred in two, leading to death from pneumocystis carinii pneumonia and to irreversible neurologic damage from listeria monocytogenes meningitis, respectively. CONCLUSIONS. We conclude that combined autoimmune cytopenias are frequently observed in patients with AIN, and a thorough search for associated conditions can lead to unsuspected diagnoses.
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ranking = 1
keywords = idiopathic
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7/11. CD4 lymphocytopenia due to common variable immunodeficiency mimicking AIDS.

    There are an increasing number of published reports of patients with acquired immunodeficiency without evidence of hiv infection, who have been labelled as having "idiopathic CD4 lymphocytopenia". The case is reported here of a young man who presented with pneumocystis carinii pneumonia (PCP), CD4 lymphopenia, and hypogammaglobulinaemia attributable to common variable immunodeficiency (CVID). The presentation of this condition, with many of the clinical and laboratory features of AIDS, highlights CVID as a diagnosis to be considered in the differential diagnosis of CD4 lymphocytopenia.
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ranking = 1
keywords = idiopathic
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8/11. Idiopathic CD4 T-lymphocytopenia--four patients with opportunistic infections and no evidence of hiv infection.

    BACKGROUND AND methods. We describe four patients without major risk factors for human immunodeficiency virus (hiv) infection, each of whom presented with severe opportunistic infections and was found to have idiopathic CD4 T-lymphocytopenia. We performed assays to detect the presence of retroviruses and undertook immunophenotyping of subgroups of peripheral-blood lymphocytes. RESULTS. The opportunistic infections at presentation included pneumocystis carinii pneumonia, cryptococcal meningitis (two patients, one with concurrent pulmonary tuberculosis), and histoplasma-induced brain abscess. During 10 to 68 months of observation, none of the four patients had evidence of infection with hiv type 1 or 2 or human T-cell lymphotropic virus type I or II on the basis of epidemiologic, serologic, or polymerase-chain-reaction studies or culture, nor was there any detectable reverse transcriptase activity. Although all the patients had severe, persistent CD4 T-lymphocytopenia (range, 12 to 293 cells per cubic millimeter), the CD4 cell count progressively declined in only one and was accompanied by multiple opportunistic infections. All four patients had significantly reduced numbers of circulating CD8 T cells, natural killer cells, or B cells (or all three). CONCLUSIONS. These four patients had idiopathic CD4 T-lymphocytopenia with opportunistic infections but no evidence of hiv infection. Instead of the progressive, selective depletion of CD4 T cells characteristic of hiv infection, some patients with idiopathic immunodeficiency have stable CD4 cell counts accompanied by reductions in the levels of several other lymphocyte subgroups.
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ranking = 3
keywords = idiopathic
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9/11. pneumocystis carinii in a patient with pulmonary sarcoidosis and idiopathic CD4 T lymphocytopenia.

    A case of pulmonary sarcoidosis and idiopathic CD4 T lymphocytopenia is reported. pneumocystis carinii was detected in the bronchoalveolar lavage fluid of a young homosexual man who was asymptomatic without any evidence of congenital or acquired immunodeficiency but with a low CD4 cell count. A clinical and histological diagnosis of pulmonary sarcoidosis was made. During follow up the patient had oral candidiasis and a CD4 cell count persistently below 300/microliters. This case is highly suggestive of concurrent pulmonary sarcoidosis and idiopathic CD4 T lymphocytopenia.
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ranking = 6
keywords = idiopathic
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10/11. Behcet's-like syndrome associated with idiopathic CD4 T-lymphocytopenia, opportunistic infections, and a large population of TCR alpha beta CD4- CD8- T cells.

    Herein we report a patient with Behcet's like syndrome, idiopathic CD4 T-lymphocytopenia, opportunistic infections, and a large polyclonal population of TCR alpha beta CD4- CD8- T cells. Microfluorimetric analysis of peripheral blood mononuclear cells revealed CD4 T-cell counts of 10 /- 5/mm3. The CD3 T cells were 99% TCR alpha beta , of which 74 /- 5% were CD4- CD8-. No clonal populations were detected by southern analysis for T-cell receptor V beta gene rearrangements. No evidence of human immunodeficiency virus infection was present, although nocardia, candida, pneumocystis, cytomegalovirus, and herpes infections were documented. The concomitant presence of opportunistic infections and a large population of TCR alpha beta CD4- CD8- T cells suggests a pathogenic association and an intense immune response to microbial lipid or lipoglycan antigens presented in the context of CD1 molecules. This case demonstrates the potential for idiopathic CD4 T-lymphocytopenia to occur in Behcet's-like syndrome with lethal consequences.
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ranking = 6
keywords = idiopathic
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