Radiology Case of the Month: Pulmonary Langerhans Cell Histiocytosis (original) (raw)

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: A Rare Case of Incipient Radiological Stage

Pulmonary Langerhans' Cell Histiocytosis (PLCH) is a rare interstitial lung disease with characteristic radiological features on high resolution computed tomography (HRCT). The diagnosis is usually delayed as the illness is either asymptomatic or has mild symptoms in the initial stage. We present a case of 54 yrs old smoker who presented with acute febrile illness associated with cough and sputum production due to upper respiratory tract infection. A subtle abnormality on chest X-ray led to the performance of HRCT chest showing few tiny nodules which progressed on repeat HRCT after a period of observation. A thoracoscopic lung biopsy confirmed the diagnosis of Pulmonary Langerhans' Cell Histiocytosis (PLCH). This case represents an earliest radiological stage of PLCH.

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...

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 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...

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.

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 ...

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.

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...