Chest
Pulmonary Alveolar Proteinosis Associated with Pneumocystis carinii: Ultrastructural Identification in Bronchoalveolar Lavage in AIDS and Immunocompromised Non-AIDS Patients
Section snippets
Patients
An ultrastructural study of the BAL fluid was performed in 37 patients. In these patients, BAL was undertaken to investigate episodes of pneumonitis. Pneumonitis was suspected because of pulmonary symptoms and/or fever and/or abnormal chest roentgenogram.
Twenty-six patients had a PC pneumonitis, 19 with AIDS and seven with an immunodeficiency related to heart (n = 3) or kidney (n = 4) transplantation fiable 1). The chest roentgenogram revealed a diffuse reticulonodular pattern in 21 patients, a
Light Microscopy
In each of the 26 cases, the identification in the BAL fluid of PC cysts by Gomori-Grocott staining confirmed the diagnosis of PC pneumonitis.
On MGG- or Papanicolaou-stained slides from BAL with PC, foamy honeycombed material containing PC cysts and trophozoites was observed in 23 of these 26 BAL fluid samples. In nine cases, an extracellular material with a different pattern was observed associated with the honeycombed material (Fig la). This extracellular material was pale, homogeneous, and
DISCUSSION
The present systematic ultrastructural investigation of a series of BAL fluid samples collected in patients with PC pneumonitis demonstrated in all of them, in addition to the aggregates of PC cysts and trophozoites, the presence of an extracellular material composed of an accumulation of phospholipids and lipoproteinaceous substance with the characteristic pattern of surfactant. This material was detected by light microscopic examination in nine cases due to the amount of the extracellular
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Update on diffuse lung disease in children
2016, ChestCitation Excerpt :Autoimmune PAP due to GM-CSF autoantibodies typically presents in older children. Secondary PAP can occur as a consequence of a number of disorders, including infections (particularly Pneumocystis jiroveci pneumonia) after hematopoietic stem cell transplantation and in association with hematologic disorders.39-43 Diagnostic methods have changed with the availability of clinical genetic testing such that genetic testing is recommended prior to lung biopsy for most cases, with the goal of obviating the need for lung biopsy.
Pulmonary Alveolar Proteinosis Syndrome
2015, Murray and Nadel's Textbook of Respiratory Medicine: Volume 1,2, Sixth EditionThe molecular basis of pulmonary alveolar proteinosis
2010, Clinical ImmunologyCitation Excerpt :The diagnosis is usually made based on the presence of typical radiological manifestations and lung histopathological findings. Secondary PAP has been reported in association with various diverse clinical disorders, including hematological disorders (myelodysplastic syndrome, leukemia, lymphoma, aplastic anemia, pharmacologically induced leukopenia, and others), immunological diseases (severe combined immunodeficiency, monoclonal gammopathy, selective immunoglobulin A deficiency, and others), lysinuric protein intolerance, and infections (Cytomegalovirus, M. tuberculosis, Nocardia, Pneumocystis jiroveci, and others) [34,62–80]. It has also been reported in association with various toxic inhalation syndromes, including inhalation of inorganic dusts (silica, cement, titanium, and aluminum), organic dusts (sawdust, fertilizer, bakery flour, and others), and fumes (chlorine, varnish, and others) [81–88].
Managing a rare condition presenting with intractable hypoxemic respiratory failure
2007, ChestCitation Excerpt :Three distinct forms of PAP exist: secondary PAP, congenital PAP, and acquired or idiopathic PAP. Environmental exposures, hematologic or neoplastic disorders, and immunodeficiency or immunologic disorders resulting in functional impairment or reduced numbers of alveolar macrophages are associated with the development of secondary PAP.2–17 Pneumocystis jiroveci, cytomegalovirus, HIV, mycobacterial, and nocardial infections are associated with PAP, but it is unclear if the infections are causative or a result of the altered macrophage function in patients with idiopathic PAP.2–8
Pulmonary alveolar phospholipoproteinosis
2005, Revue de Pneumologie CliniquePulmonary alveolar proteinosis
2004, Pathology Research and PracticeCitation Excerpt :The pathogenesis of PAP remained an enigma for decades. A defect in alveolar clearance and/or alveolar macrophage activity associated with an overproduction of lipid by type 2 alveolar lining cells has been suggested [7,16,20–22]. Most data suggest that the macrophage defect in PAP is secondary, as alveolar macrophages and type 2 cell clearance mechanisms are overwhelmed by the accumulation of the surfactant-rich material, and this leads to impaired phagocytosis and phagolysome fusion [21].
This investigation was supported by Crédits Universitaires, Université Paris XII, France.
Manuscript received October 20; revision accepted March 29.