Archivos de Bronconeumologia http: www. Other types of articles such as reviews, editorials, special articles, clinical reports, and letters to the Editor are also published in the Journal. It is a monthly Journal that publishes a total of 12 issues, which contain these types of articles to different extents. All manuscripts are sent to peer-review and handled by the Editor or an Associate Editor from the team.
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This article focuses on the latter. PLCH is usually identified in young adults years of age. It is a rare disorder with no well-established gender predilection, which appears to be more common in Caucasian populations 4.
Presentation is usually with dyspnea or a non-productive cough. Other symptoms include constitutional symptoms fatigue and weight loss , pleuritic chest pain, or spontaneous pneumothorax 1,4. Up to a quarter of patients are asymptomatic. It is postulated that as these cellular granulomas evolve, peripheral fibrosis forms resulting in traction on the central bronchiole which becomes cyst-like 3.
This explains the presumed evolution from nodule, through cavitating nodule and thick walled cysts, to the 'stable' thin-walled cysts 3,4. An immune-mediated mechanism has been postulated, although an inciting agent has not been isolated 4. This proliferation is accompanied by inflammation and granuloma formation.
Electron microscopy may reveal characteristic Birbeck granules 1,2. More recent evidence suggests that PLCH represents a myeloid neoplasm with inflammatory properties 9. Pulmonary Langerhans cell histiocytosis has variable appearance depending on the stage of disease, ranging from small peribronchiolar nodular opacities to multiple irregularly-shaped cysts.
There is a mid and upper zone predilection 1,3,4. The earliest change is a diffuse bilateral symmetrical reticulonodular pattern with a predilection for the mid and upper zones. The ill-defined nodules range from mm in size. Later, cyst formation may be seen or may mimic a honeycomb appearance due to a summation of air-filled cysts. There is a preservation of lung volumes or even hyperinflation 1,3,4.
Reduced lung volumes are uncommon and only seen in end-stage fibrotic cases 4. Lymph node enlargement visible on chest x-rays is rare 4. As is usually the case, CT and especially HRCT is superior to plain chest radiography in identifying both the reticulonodular opacities and cysts 1,3,4. Distribution is the key in differentiating PLCH from other cystic lung diseases with predilection for the mid and upper zones and regional sparing of the costophrenic recesses, anterior right middle lobe and lingula left upper lobe 1,3,4.
The appearance of new nodules later in the disease when cystic change is established indicates disease progression but is a rare finding 3. This is especially the case in patients who stop smoking. Treatment may not be required once smoking has ceased.
Corticosteroids are frequently used and appear beneficial. In patients with rapidly progressive disease, no proven therapy has been found. In some selected patients lung transplantation may be an option, provided smoking has ceased. Recurrence in the transplanted lung has been described 4. See differential of multiple pulmonary nodules and differential of miliary opacities for more comprehensive lists.
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Radiology abstract [ pubmed citation ] 4. Pulmonary histiocytosis X: comparison of radiographic and CT findings. Radiology abstract - Pubmed citation. Promoted articles advertising. Edit article Share article View revision history Report problem with Article. URL of Article. Article information. System: Chest. Tag: infectiousdisease. Support Radiopaedia and see fewer ads. Cases and figures. Figure 1: illustration Figure 1: illustration. Case 1 Case 1. Case 2 Case 2.
Case 3: chest radiograph Case 3: chest radiograph. Case 4: chest radiograph Case 4: chest radiograph. Case 5 Case 5. Case 6 Case 6. Case 8 Case 8. Case 10 Case Case 11 Case Case early nodular stage Case early nodular stage. Case 13 Case Case with pneumothorax Case with pneumothorax. Case 15 Case Case 16 Case Imaging differential diagnosis. Lymphangiomyomatosis Lymphangiomyomatosis. Lymphocytic interstitial pneumonitis Lymphocytic interstitial pneumonitis. Loading more images Close Please Note: You can also scroll through stacks with your mouse wheel or the keyboard arrow keys.
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Histiocitosis X pulmonar: Presentacin de un caso y revisin de la literatura
As idades extremas 18 e 61 anos foram de pacientes do sexo feminino. Dois destes sete pacientes eram ex-tabagistas. As paredes foram referidas como finas, quando menores que 1 mm, e espessas, quando maiores que 1 mm. Uma das seis radiografias foi considerada normal. No entanto, a TCAR desta paciente mostrou raros cistos de paredes finas. Infiltrado reticular foi observado em quatro casos e reticulonodular, em um. Em quatro casos, no entanto, notamos, associada aos cistos de paredes finas, pequena quantidade de cistos de paredes espessas, com mais de 1 mm Figura 4.
Langerhans cell histiocytosis
Nezelof C, Basset F. Langerhans cell histiocytosis. Hematol Oncol Clin North Am ; Contemporary classification of histiocytic disorders. Med Pediatr Oncol Bravencova E, Abdellatif T et al.