Adult pulmonary Langerhans' cell histiocytosis (original) (raw)
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Bronchoalveolar Lavage as a Diagnostic Tool in an Atypical Pulmonary Langerhans Cell Histiocytosis
Diagnostics
Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon diffuse cystic lung disease that occurs almost exclusively in young adult smokers. High-resolution computed tomography of the chest allows a confident diagnosis of PLCH in typical presentation, when nodules, cavitating nodules, and cysts coexist and show a predominance for the upper and middle lung. Atypical presentations require histology for diagnosis. Histologic diagnosis rests on the demonstration of increased numbers of Langerhans cells and/or specific histological changes. PLCH is one of the few diseases in which bronchoalveolar lavage (BAL) has a high diagnostic value and can in some circumstances replace lung biopsy. We present a case of PLCH in an elderly non-smoker. Chest imaging revealed the presence of advanced interstitial lung disease with a fibrocystic pattern. BAL cellular analyses disclosed a macrophage pattern with CD1a phenotype that strongly supports the PLCH diagnosis, even in the setting of atypical ...
Pulmonary Langerhans Cell Histiocytosis (Histiocytosis X) on Bronchoalveolar Lavage
Acta Cytologica, 2007
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial lung disease seen as part of multisystem Langerhans cell histiocytosis, or as an isolated form of the disease. The majority of cases of the latter are seen in adults and associated with cigarette smoking, which probably plays a central etiologic role. Although the mechanisms leading to the development of isolated PLCH are unclear, it is thought to be a reactive process in contrast to other forms of Langerhans cell histiocytosis, which have been shown to represent clonal, neoplastic proliferations of Langerhans cells. Isolated PLCH and the other forms may be altogether separate diseases, but they have in common great variability in severity and, likewise, unpredictable clinical courses. In this article, we provide an historical context for these elusive diseases, as well as summarize the most recent literature on PLCH. FIGURE 4. Fifty-three-year-old woman with a 30 pack-year history of smoking presented with chronic dyspnea and cough. Chest radiograph (A, B) and CT (C, D) show cysts of varying wall thickness and shape with relative sparing of the lower lung fields. PLCH was diagnosed on surgical biopsy.
VATS biopsy of an adult with pulmonary langerhans cell histiocytosis: case report
2020
A 29-year-old Filipino male smoker presented with dyspnea and a right-sided pneumothorax with subsequent chest tube drainage. Chest CT scan revealed multiple diffuse and mostly interconnected thin and thick-walled cystic structures of varying sizes and shapes with most located in both upper lobes. Video-assisted thoracoscopic surgery (VATS) wedge resection of the right lower lobe posterobasal segment and mechanical pleural abrasion were done. Intraoperatively, there were multiple bullae and diffuse small cysts interspersed with normal lung tissue. Histopathology and immunohistochemistry findings revealed pulmonary Langerhans cell histiocytosis (PLCH). Smoking has been hypothesized to induce a clonal proliferative, neoplastic process or a reactive immune activity in PLCH. The patient was advised smoking cessation and is on close follow-up. He is doing well 10 months after hospital discharge. PLCH is a very rare disease and diagnosis was obtained by VATS. To the authors’ knowledge, th...
An Elderly Smoker Presenting With Pulmonary Langerhans Cell Histiocytosis: An Unusual Demographic
Cureus, 2021
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare neoplastic condition that occurs almost exclusively in young smokers and presents with multiple solid and/or cystic nodules in a primarily upper lobe distribution on chest imaging. Frequently, suspicion arises incidentally on imaging performed for alternative reasons, such as lung cancer screening, but diagnosis requires biopsy studies. We describe an uncommon case of PLCH in an elderly female patient presenting with mild dyspnea and a significant history of smoking. Diagnosis was made with a biopsy of a new pulmonary nodule in her left lung found on lung cancer screening computed tomography (CT) scan. No gold standard therapy exists, and novel agents are being studied for future use. At present, she has been advised to quit smoking and is being followed with serial imaging studies to determine additional measures.
Radiology Case of the Month: Pulmonary Langerhans Cell Histiocytosis
The Journal of the Louisiana State Medical Society: official organ of the Louisiana State Medical Society
Purpose: Obtaining a tissue sample diagnostic of pulmonary Langerhans cell histiocytosis (PLCH) by trans-bronchial biopsy is notoriously difficult. The condition’s appearance on computed tomography is well described and singularly characteristic, perhaps adequate for definitive diagnosis. We propose an approach to diagnosis of these patients. Methods: Radiology case report of PLCH in a middle-aged female smoker with two week history of nonpro-ductive cough, low grade fevers, and fatigue. Results: Computed tomography (CT) provided the diagnosis of PLCH. Transbronchial biopsies failed to provide a definitive diagnosis. Conclusions: Utility of transbronchial biopsy in diagnosis of PLCH is limited. Patients who present with signs, symptoms and high resolution computed tomography typical of PLCH do not require a correlation by tissue diagnosis. If cancer is suspected, a wedge biopsy should be performed for tissue diagnosis.
Pulmonary langerhans cell histiocytosis: Two cases with varied radiologic findings
Eastern Journal Of Medicine
Pulmonary Langerhans Cell Histiocytosis (PLCH) is an idiopathic interstitial lung disease with Langerhans cell infiltration in the lung. PLCH X has non-spesific symptoms, and most patients have smoking history. A combination of stellate nodules, reticular and nodular opacities, upper zone cysts or honeycombing, preservation of lung volume and costophrenic angle sparing are highly specific for PLCH. To contribute to the literature, two cases are presented. First case is 30 years old man with 10 pack/years smoking history was admitted with cough and persevering interstitial opacities. Second case is 34 years old man with 15 pack/years smoking history was admitted with persistant cough. On thorax CT the first case had reticulonodular opacities at the perifery of the upper and middle zones, second case had multiple parenchymal cystic nodular lesions. Open lung biopsy performed, immunohistochemical examination CD1a, CD68, S100 detecting antigenpositive and histologically and radiologically confirmed diagnosed of PLCH.
Pulmonary Langerhans cell histiocytosis in a 26-year-old female: still a diagnostic challenge
Romanian journal of morphology and embryology = Revue roumaine de morphologie et embryologie, 2017
Langerhans cell histiocytosis (LCH) is a rare disorder caused by monoclonal Langerhans cells proliferation in bone, skin, lung, lymph nodes, liver, spleen, nervous or hematopoietic system. Pulmonary LCH is a diagnostic trap that is displayed on computed tomography (CT) as an interstitial disorder with honeycomb aspect. In this paper, we present an unusual case of a 26-year-old female that was hospitalized with progressive worsening dyspnea and history of recurrent pneumonia. Lung biopsy showed fibrosis of the interalveolar septa, architectural distortion and large cells with foamy cytoplasm and convoluted nuclei that were marked by CD68, S-100 and the specific antibody CD1a that allowed establishing the diagnosis of pulmonary LCH. The only extrapulmonary manifestations were femoral bone cysts that were radiologically seen 10 years before and were not modified along the years. The therapy consisted on smoking cessation and oral corticosteroids without significant improvement of the c...
Journal of Nippon Medical School = Nippon Ika Daigaku zasshi, 2009
Histiocytes of Langerhans cell type are recovered from the bronchoalveolar lavage fluid (BALF) of patients with interstitial lung diseases in a nonspecific manner. Langerhans cells (LCs) can be identified through immunostaining for S-100, CD1a, and, more specifically, langerin. To evaluate the diagnostic value of BALF in pulmonary Langerhans cell histiocytosis (PLCH), we performed a retrospective clinicopathological study of 5 patients with biopsy-confirmed PLCH or Hand-Schüller-Christian disease involving the lung. As a control study, we examined BALF cells from 23 patients with various diseases, including sarcoidosis, hypersensitivity pneumonitis, collagen vascular disease, idiopathic pulmonary fibrosis, and adenocarcinoma of the lung. Cytospins obtained from BALF were stained with Giemsa or Papanicoloau and others were immunostained. In general, cytospins showed a monomorphous and dispersed cell population containing mononucleated or binucleated and occasionally multinucleated hi...
Pulmonary Langerhans cell histiocytosis: the many faces of presentation at initial CT scan
Insights into imaging, 2014
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial granulomatous disease that usually affects young adults who are smokers. Chest computed tomography (CT) allows a confident diagnosis of PLCH only in typical presentation, when nodules, cavitated nodules and cysts coexist and predominate in the upper and middle lungs. This article includes a pictorial essay of typical and atypical presentations of PLCH at initial chest CT. Various appearances of PLCH are illustrated and possible differential diagnosis is discussed. PLCH can present with some aspecific features that may cause diagnosis of the initial disease to be overlooked or other pulmonary diseases to be suspected. In cases of nodule presentation alone, the main differential diagnosis should include lung metastasis, tuberculosis and other infections, sarcoidosis, silicosis and Wegener's disease. In cases of cysts alone, the most common diseases to be differentiated are centrilobular emphysema and lymphangiom...
General features of patients with Pulmonary Langerhans Cell Histiocytosis followed in our instution
Tuberkuloz ve toraks, 2018
Introduction Pulmonary Langerhans Cell Histiocytosis (PLCH) is a rare disease affecting young smokers. It is more common between the ages of 20-40 and equals the male/female ratio. Lung biopsy is the most useful methods for diagnosis. The first treatment is to quit smoking. Corticosteroids or chemotherapeutic agents can be used in severe progressive cases despite of quiting smoking. The patients with PLCH followed in our clinic were assessed with general clinical features in the light of the literature. Materials and Methods We retrospectively evaluated patients with PLCH in our clinic between January 1999 and June 2017. Result The female and male distribution of the 21 patients was 11/10. The average age was 35.04 ± 11.78 years. All patients were active smokers at the time of admission. The most common symptom was dyspnea. The most common finding in the pulmonary function tests was obstructive ventilatory defect. The DLCO value of the 70% patient in the carbonmonooxid diffusion tes...