VATS biopsy of an adult with pulmonary langerhans cell histiocytosis: case report (original) (raw)
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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.
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.
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 ...
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.
Adult pulmonary Langerhans' cell histiocytosis
European Respiratory Journal, 2006
Adult pulmonary Langerhans' cell histiocytosis is a rare disorder of unknown aetiology that occurs predominantly in young smokers, with an incidence peak at 20-40 yrs of age. In adults, pulmonary involvement with Langerhans' cell histiocytosis usually occurs as a single-system disease and is characterised by focal Langerhans' cell granulomas infiltrating and destroying distal bronchioles. High-resolution computed tomography (HRCT) of the chest is essential to the diagnosis, typically showing a combination of nodules, cavitated nodules, and thick-and thin-walled cysts. A high macrophage count in bronchoalveolar lavage (BAL) fluid is a common but nonspecific finding that merely reflects exposure to tobacco smoke. BAL is useful for eliminating infections and the other infiltrating lung disorders that can be seen in young adults. Langerhans' cells can be identified in BAL fluid, but, in contrast to what was initially hoped, this test shows a very low sensitivity and is rarely useful in the diagnosis of the disease. The definite diagnosis of pulmonary Langerhans' cell histiocytosis requires identification of Langerhans' cell granulomas, which is usually achieved by surgical lung biopsy at a site selected by chest HRCT. In practice, however, lung biopsy is performed on a case-by-case basis. No effective treatment is available to date, and improved understanding of the mechanisms involved in the pathogenesis of pulmonary Langerhans' cell histiocytosis is urgently needed, and should help in the development of specific therapeutic strategies for patients with this orphan disease.
Indian journal of child health, 2020
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disease in childhood. We report a case of a 3-year-old boy who was exposed to chronic passive smoking in family presented with PLCH with isolated skin involvement. The boy presented with a history of recurrent respiratory tract infections requiring intensive care unit admissions for severe respiratory distress. The chest X-ray showed bilateral honeycombing of lungs, which was further confirmed by chest computed tomography showing diffused bilateral multiple cystic lesions with ground-glass haziness of lung parenchyma. Skin biopsy from suspected cutaneous lesions confirmed the diagnosis by positive S-100 and CD-1a. After initiating intensive chemotherapy, the patient showed significant improvement. Based on our experience, it can be suggested that knowledge of such condition with high clinical suspicion and simple skin biopsy can be a valuable modality in diagnosing PLCH in cases without putting the other internal organs at risk.
The Journal of medical research, 2018
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare pulmonary disorder with characteristic imaging features. It usually affects young adults and is associated with cigarette smoking. PLCH is listed in the gamut for cystic lung disease and should be considered by the radiologist if the appropriate findings are identified. The authors report an advanced case of PLCH in a young male with a smoking load of 6 pack-year. The prognosis is variable with frequent regression, stabilization, or recurrence of disease. GJMR-D Classification: NLMC Code: WI 800 PulmonaryLangerhansCellHistiocytosisinaMaleTeenagerACaseReportwithEmphasisonHRCTFindings Strictly as per the compliance and regulations of: Radiology, Diagnostic and Instrumentation Pulmonary Langerhans Cell Histiocytosis in a Male Teenager A Case Report with Emphasis
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...
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.