Bronchiolocentric pulmonary involvement due to chronic lymphocytic leukemia.
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Biomedical subjects
Publications and source records attributed to V Poletti.
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Idiopathic interstitial pneumonias are defined from the pathological point of view as non granulomatous intralobular inflammatory and fibrotic processes involving the alveolar walls. More than thirty years ago Liebow and Carrington pioneered the notion that morphological characteristics could be used with benefit in separating the different entities found in this group, which present with typical, but not pathognomonic clinical features. In the mid-1980s some entities, including giant cell interstitial pneumonia (GIP) and lymphocytic interstitial pneumonia (LIP), were removed from this group and considered as peculiar forms. In the early 90s the concept of cellular or nonspecific interstitial pneumonia was reconsidered, leading to an in depth revision of various types of interstitial pneumonia of unknown etiology. The histological pattern observed in patients with idiopathic pulmonary fibrosis is now referred to as usual interstitial pneumonia (UIP). Other entities that have been revised during the last ten years are desquamative interstitial pneumonia/alveolar macrophage pneumonia (DIP/AMP), respiratory bronchiolitis-interstitial lung disease (RB-ILD), acute interstitial pneumonia (AIP), cryptogenic organizing pneumonia (COP) and nonspecific interstitial pneumonia (NSIP). This paper provides a detailed description of pulmonary disorders which have been included in the new classification systems of idiopathic interstitial pneumonias. In the second part of the paper we will discuss several doubts and controversies that this new classification schemes leave unresolved.
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PURPOSE: Nongastrointestinal locations represent about 30% to 40% of all low-grade mucosa-associated lymphoid tissue (MALT) lymphomas. We report a retrospective analysis of 75 patients with nongastrointestinal low-grade MALT lymphoma, presenting their clinical, therapeutic, and follow-up data with respect to the initial location of the lymphoma. PATIENTS AND METHODS: From January 1988 to October 1997, 75 patients with untreated nongastrointestinal low-grade MALT lymphoma were subjected to treatments ranging from local radiotherapy and local interferon alfa administration to chemotherapy. The lymphomas were located in the lung (19 patients), orbital soft tissue (16 patients), skin (seven patients), thyroid (seven patients), lachrymal gland (six patients), conjunctiva (six patients), salivary gland (six patients), breast (three patients), eyelid (two patients), larynx (one patient), bone marrow (one patient), and trachea (one patient). RESULTS: Complete and partial remissions were achieved in 59 (79%) and 16 (21%) of the 75 patients, respectively, with an overall response rate of 100%. All but two of the patients are still alive, with a median follow-up of 47 months; these two patients died from other causes. The estimated time to treatment failure rate is 30% at 5 years. In the thyroid and lachrymal gland sites, no relapses were reported. CONCLUSION: Our data regarding the largest reported series of nongastrointestinal MALT lymphomas confirm the good prognosis of this particular clinicopathologic entity and the significant efficacy of different therapeutic approaches to specific sites.
In order to assess the diagnostic yield of bronchoalveolar lavage (BAL) and transbronchial lung biopsy (TBB) in pulmonary diseases with a ground-glass and/or alveolar pattern on high-resolution computed tomography (HRCT) scan, a prospective study was undertaken. Thirty-six patients (17 males, 19 females), mean age 53 yrs, selected on the basis of the presence of an alveolar and/or ground-glass pattern on chest HRCT scan, were submitted to fibreoptic bronchoscopy. All patients underwent BAL. TBBs were performed in 33 cases. A specific diagnosis was achieved, taking into account data obtained by means of serology, microbiology, cytology and histopathology in appropriate clinical settings. Twelve (33%) patients only had the appearance of a ground-glass opacity, whereas 24 (67%) had associated areas of airspace consolidation. BAL was performed in all cases and gave a definitive diagnosis in 21 (58%). The diagnostic yield of BAL in patients with only ground-glass opacities was no different from that in those patients also showing areas of alveolar consolidation (58 versus 58%). In eight patients (six with ground-glass opacity and two with alveolar consolidation), BAL provided useful but not definitive information. In these patients, a definitive diagnosis was achieved by means of TBB in seven cases and by open lung biopsy in one case. TBB was performed in 33 out of 36 patients and gave positive results in 25 (76%). The diagnostic yield of TBB in patients showing areas of alveolar consolidation was significantly higher than in those with pure ground-glass opacity, i.e. 95% (21 of 22) and 36% (4 of 11) respectively (p < 0.001). BAL and TBB were performed during the same bronchoscopy in 33 patients, and an accurate diagnosis was achieved in 30 (91%). Overall, the diagnostic yield of TBB (76%) and BAL (56%) did not differ significantly in the whole patient group (p = 0.12), or in patients with a ground-glass opacification (58 versus 36%, p = 0.3). However, in patients with areas of alveolar consolidation, the diagnostic sensitivity of TBB (95%) was significantly greater than the diagnostic sensitivity of BAL (54%) (p = 0.03). In conclusion this study shows that high-resolution computed tomography can be helpful in predicting the diagnostic accuracy of bronchological procedures, in particular of bronchoalveolar lavage and transbronchial biopsy, and that alveolar and/or ground-glass are favourable patterns for these diagnostic tools.
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Photodynamic therapy (PDT) is an effective modality that can be used in the treatment of bronchogenic squamous cancer. PDT can be curative in cases of early-stage central type lung cancer and, for this reason, is an alternative to surgery in patients with in situ carcinoma or minimally invasive squamous cell carcinoma. PDT was performed in 26 early bronchogenic cancers in 23 patients, and 16 complete remissions and 10 partial remissions were obtained. It is concluded that photodynamic therapy is effective in the treatment of superficial lung cancer in which complete remission can be expected. The combination of different endoscopic therapeutic modalities and trials comparing endoscopic therapy with surgical resection are anticipated.
Inflammation involving the small airways is a quite common report in pathological dissertations. However the radiologic, clinical patterns and functional impairment of adult bronchiolitis have been discussed in detail only in the last ten years. In this review a brief summary of the anatomic and histologic characteristic of small airways is reported. A pathologic classification of bronchiolitis is at first discussed. Cellular bronchiolitis, proliferative bronchiolitis with or without intraalveolar loose fibrosis (BOOP pattern), occlusive and constrictive bronchiolitis are the main patterns taken into account: peculiar subtypes (follicular bronchiolitis, diffuse panbronchiolitis, neuroendocrine cell hyperplasia with fibrous bronchiolitis) are included in the pathologic discussion. Radiologic features are reported and presented as nonspecific. HRCT Scan findings are classified with the appropriate pathologic features in: nodules and branching lines; low attenuation and mosaic perfusion; ground glass attenuation and/or alveolar consolidation. The clinical entities considered are: bronchiolitis secondary to irritant inhalation; infectious and post-infectious bronchiolitis; drug induced bronchiolitis; bronchiolitis in patients with collagen-vascular disease; diffuse panbronchiolitis; bronchiolitis in transplanted patients; neuroendocrine cell hyperplasia with fibrous bronchiolitis: cryptogenic bronchiolitis; idiopathic BOOP; respiratory bronchiolitis with interstitial lung disease (RB-ILD). Their clinical presentation, functional impairment, pathogenetic mechanisms when deemed clinically useful, BAL findings and therapeutical schemes are discussed.
A case of cervicofacial and pulmonary actinomycosis associated with non-Hodgkin's lymphoma (NHL) is reported. The patient underwent antimicrobial treatment and surgical debridement of a palatine lesion. Complete clinical recovery was achieved. The presence of actinomycosis may obscure and delay the diagnosis of NHL.
BACKGROUND: Peripheral B-cell lymphoma of the marginal zone (MALT, low-grade), presenting as localized, extranodal disease, usually affects the elderly. The gastrointestinal tract is the most frequently involved extranodal location, representing 70% of all MALT lymphomas. Recently, numerous other extranodal sites involved by MALT lymphomas have also been described. PATIENTS AND METHODS: From January 1990 to October 1995, 24 patients with untreated nongastrointestinal low-grade MALT lymphoma were submitted to treatments ranging from the local approach of radiotherapy and local alpha-interferon (alpha-IFN) administration to chemotherapy. The tumours were located in the lung (seven cases), conjunctiva (four cases), lachrymal gland and orbital soft tissue (four cases), salivary glands (three cases), skin (three cases), breast (two cases), and thyroid (one case). All patients had low-grade stage IE tumours. RESULTS: Chemotherapy was administered in 11 patients (six with lung, three with salivary gland, one with breast, and one with thyroid locations); radiation therapy was employed in seven patients (three with lachrymal gland, three with skin, and one with breast locations); local alpha-IFN administration was administered in five patients (four with conjunctival, and one with lachrymal gland sites); and surgery was employed in one patient with a lung tumour. All patients achieved complete remissions; three local recurrences and two relapses in other sites were observed. The global five-year survival rate was 100% with a relapse-free survival rate of 79%. CONCLUSIONS: These data confirm the significant efficacy of different therapeutic approaches to specific sites inbes obtaining a good remission rate for nongastrointestinal localized low-grade MALT lymphomas.
Bronchioles are the airways less than 2-3 mm in diameter. Normal bronchioles cannot be reliably detected by means of high resolution computed tomography (HRCT). Nevertheless, in pathological cases, bronchiolar lesions can be identified by taking into account direct and indirect signs. On radiological grounds, bronchiolar lesions can be classified into four groups, on the basis of HRCT findings: 1) prevailing nodular opacities and "tree in bud" pattern; 2) consolidations or ground-glass opacities; 3) mosaic oligosemia with expiratory air-trapping; and 4) mixed cases. In this review, we present the main radiological and HRCT findings in four different entities, representing the more typical cases of bronchiolar pathology; bronchiolitis obliterans; bronchiolitis obliterans with organizing pneumonia; diffuse panbronchiolitis; and respiratory bronchiolitis with associated interstitial lung disease. HRCT sometimes allows a precise diagnosis of bronchiolar pathology; however, more often, it permits only a range of hypotheses to be advanced. More importantly, it allows a precise localization for biopsy procedures and an exact follow-up after institution of therapy. In normal subjects, less than 1% of the whole bronchial tree is visible on the standard chest radiograph. HRCT offers a good insight and invaluable information. New techniques, such as volumetric HRCT with sliding-thin-slab maximum- and minimum-intensity projections (MIP and minip) could represent an important additional tool in the evaluation of small airways disease.
A young male who was a heavy smoker presented with spontaneous right pneumothorax. A high resolution computed tomography scan showed disseminated nodules up to 1 cm in diameter; the greatest majority of which were sited in the centrilobular zone, though some abutted on the pleural surface. Surgical lung biopsies allowed a diagnosis of epithelioid haemangioendothelioma. The neoplastic tissue infiltrated the wall of bronchioles, partially obliterating them and the visceral pleura. These two histological aspects could be considered as concomitant mechanisms for the appearance of spontaneous pneumothorax. Epithelioid haemangioendothelioma should be added to the list of lung diseases in young heavy smokers that can begin with a spontaneous pneumothorax.
Obliterative or constrictive bronchiolitis is characterized by narrowing of the small airways, due to submucosal and peribronchiolar fibrosis, with chronic obstruction. The vast majority of cases of bronchiolitis obliterans are associated with other diseases and only few cases are idiopathic. We report on the main computed tomography (CT) methods used study obliterative bronchiolitis, the CT findings and the differential diagnosis with other diseases. The dynamic study of alveolar ventilation with CT uses inspiratory and expiratory CT or high-resolution CT (HRCT), spiral dynamic CT or HRCT with advanced image display, ultrafast CT. In abnormal cases HRCT shows direct and indirect signs of small airways disease. The most common (> 80%) sign of obliterative bronchiolitis is the so-called mosaic oligohemia, with low attenuating lobules, caused by air trapping and best seen on expiratory CT, associated with blood flow redistribution to more normal lobules; this finding simulates the ground-glass pattern from infiltrative lung disease. Differential diagnosis is more difficult in the presence of true ground-glass patterns associated with diffuse bronchiolar obstruction and also with mosaic oligohemia due to pulmonary vascular disease and pulmonary emphysema. HRCT can distinguish these diseases and dynamic CT is more sensitive than functional tests in detecting regional abnormalities and air trapping. The combination of HRCT, rapid volumetric scanning and advanced image display is a powerful tool study the normal and abnormal features of bronchiolar function and alveolar ventilation.
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In patients with idiopathic alveolar proteinosis, the alveoli are filled with materials rich in surfactant components, especially surfactant protein A (SP-A). The anomaly could be caused by either increased secretion, decreased clearance, or both. To clarify this point, we studied five patients who underwent therapeutic lavage and then were ventilated mechanically for 24 h. During the first 8 h of mechanical ventilation, a surfactant-depleted lung was lavaged at selected intervals, and the bronchoalveolar lavage fluid was analyzed. We observed that, after lavage, various surfactant components accumulated in the airways with different time courses. We also observed that SP-A increased until the second hour and then dropped rapidly, suggesting the existence of an efficient mechanism of removal. These findings suggest that idiopathic alveolar proteinosis might be caused by a primary defect in a slow mechanism of removal or by the presence of factor(s) that interfere with the clearance of surfactant and that can be removed by lavage. It seems clear, however, that an increased secretion rate is unlikely to be the major cause of idiopathic alveolar proteinosis.
In order to determine the diagnostic value of bronchoalveolar lavage (BAL) and transbronchial lung biopsy (TBLB) in cryptogenic organizing pneumonia (COP) a prospective study was carried out. Thirty seven consecutive patients (20 males and 17 females) with clinicoradiological features of COP were enrolled in the study. The statistical analyses were completed in 35 cases. Twenty eight patients were diagnosed to have COP, all of them with a confirmatory biopsy. In seven cases, a different diagnosis was made. BAL cytological and phenotypical criteria considered for the diagnosis of COP were: a lymphocytosis of more than 25% (with a CD4/CD8 ratio less than 0.9); combined with at least two of the following data (foamy macrophages of > 20%, and/or neutrophils of > 5%, and/or eosinophils of > 2% and < 25%). TBLB specimens were classified as positive for COP if they showed: buds of granulation tissue within the centrilobular air spaces; infiltration of alveolar walls with chronic inflammatory cells; and preservation of alveolar architecture. BAL was performed in 34 patients; 17 cases were consistent with the final diagnosis of COP (sensitivity 63%), and four cases were correctly classified as negative (specificity 57%). BAL had a positive predictive value (PPV) of 85% and a negative predictive value (NPV) of 29%. TBLB was performed in 32 patients; it correctly identified COP in 16 cases (sensitivity 64%), and six cases were correctly classified as negative (specificity 86%). TBLB had a PPV of 94% and a NPV of 40%. The accuracy of the examinations, that is the probability of correctly diagnosing both diseased and nondiseased patients by BAL or TBLB, was 62 and 69%, respectively. Our findings suggest that the combination of cytological bronchoalveolar lavage and histological transbronchial lung biopsy data obtained during a fibreoptic procedure appears to be an effective method for the initial investigation in cryptogenic organizing patients pneumonia presenting with patchy radiographic shadows.
The authors report the results of the study performed with high resolution CT (HRCT) in a group of 29 patients affected with idiopathic pulmonary fibrosis (IPF). Each patient underwent HRCT at the beginning of the study and after one year. A complete clinico-functional assessment was available in 20 cases and functional CT correlation was made in these patients; 15/20 subjects underwent immunosuppressive therapy with corticosteroids and cyclophosphamide. Disease severity was assessed with chest radiography and HRCT. On the basis of CT findings the patients were classified into three groups, according to Wells classification: predominant ground-glass pattern, mixed pattern and predominant reticular disease with honeycombing. Furthermore, a visual score was assigned to total disease extent and a different score to ground-glass and reticular opacities. Our data confirm the poor accuracy of chest radiography in assessing disease type and overall severity, versus the outstanding diagnostic accuracy of HRCT. We also found a high incidence of mediastinal adenopathies (37.9% of the patients) and signs of pulmonary arterial hypertension (62%), together with low extent of pulmonary emphysema (65.5% of the patients; mean extent: 5.4%). Ground-glass attenuation is an early sign of IPF and might suggest alveolitis activity. In our series, however, the patients with grade I disease were rare (6.8%), but likely to benefit from therapy. Different from Wells, we found no significant difference in the evolution of the patients with grade II versus grade III disease. Wells grading was useful in early disease assessment, but the visual score of total disease extent and the score of ground-glass and reticular opacities were much more useful in the follow-up because they can assess disease progression. Furthermore, in the few patients with predominant alveolitis, who improve after therapy, the relative prevalence of the reticular pattern might allocate the patient in a higher Wells group with a "paradoxical" worsening, if the visual score of the extent of the primary lesion is not used.
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