Pulmonary Langerhans' Cell Histiocytosis: A Rare Case of Incipient Radiological Stage (original) (raw)
Related papers
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...
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.
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.
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...
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
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...
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.
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.