scholarly journals Pulmonary Langerhans Cell Histiocytosis and Diabetes Insipidus in a Young Smoker

2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
K. Earlam ◽  
C. A. Souza ◽  
R. Glikstein ◽  
M. M. Gomes ◽  
S. Pakhalé
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Pulmonary Involvement ◽  
Langerhans Cell ◽  
Cytotoxic Agents ◽  
Current Cigarette Smoking ◽  
System Disease ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Multisystem Involvement ◽  
Organ Systems ◽  
Current Cigarette

Langerhans cell histiocytosis is characterized by the abnormal nodular proliferation of histiocytes in various organ systems. Pulmonary involvement seen in young adults is nearly always seen in the context of past or current cigarette smoking. Although it tends to be a single-system disease, extrapulmonary manifestations involving the skin, bone, and hypothalamic-pituitary-axis are possible. High resolution CT (HRCT) of the thorax findings includes centrilobular nodules and cysts that are bizarre in shape, variable in size, and thin-walled. Often the diagnosis can be made based on the appropriate clinical presentation and typical imaging findings. Treatment includes smoking cessation and the potential use of glucocorticoids or cytotoxic agents depending on the severity of disease and multisystem involvement.

Pulmonary Langerhans Cell Histiocytosis and Other Pulmonary Histiocytic Diseases: A Review

2008 ◽  
Vol 132 (7) ◽  
pp. 1171-1181 ◽  
Author(s):  
Timothy Craig Allen
Keyword(s):  
Literature Review ◽  
Langerhans Cell Histiocytosis ◽  
Correct Diagnosis ◽  
Pulmonary Involvement ◽  
Langerhans Cell ◽  
Data Sources ◽  
Primary Material ◽  
Pulmonary Langerhans Cell Histiocytosis

Abstract Context.—Pulmonary Langerhans cell histiocytosis is the most common and best known pulmonary histocytic lesion; however, the realm of pulmonary histiocytic lesions also includes an assortment of uncommon diseases that may exhibit pulmonary involvement. Objective.—To review pulmonary Langerhans cell histiocytosis and other pulmonary histiocytoses to better ensure correct diagnosis and optimal assessment of prognosis and treatment. Data Sources.—Literature review and primary material from the author's institution. Conclusions.—This review discusses the most common pulmonary histocytosis, pulmonary Langerhans cell histiocytosis, and also reviews the uncommon pulmonary histiocytic lesions, which are distinct from pulmonary Langerhans cell histiocytosis.

scholarly journals Rosai-Dorfman Disease: Rare Pulmonary Involvement Mimicking Pulmonary Langerhans Cell Histiocytosis and Review of the Literature

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Rashid AL Umairi ◽  
Danielle Blunt ◽  
Wedad Hana ◽  
Matthew Cheung ◽  
Anastasia Oikonomou
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Corticosteroid Treatment ◽  
Pulmonary Involvement ◽  
Skin Lesions ◽  
Langerhans Cell ◽  
Sinus Histiocytosis ◽  
Review Of The Literature ◽  
Massive Lymphadenopathy ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Rosai Dorfman Disease

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare nonmalignant lymphohistiocytic proliferative disorder. We report a patient with RDD who presented with multiple skin lesions, pulmonary involvement, and CT manifestations mimicking Langerhans cell histiocytosis, which improved after initiation of corticosteroid treatment.

scholarly journals Adult Langerhans Cell Histiocytosis with Hepatic and Pulmonary Involvement

2015 ◽  
Vol 2015 ◽  
pp. 1-4
Author(s):  
Bruno Araujo ◽  
Francisco Costa ◽  
Joanne Lopes ◽  
Ricardo Castro
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Early Stage ◽  
Biliary Tree ◽  
Pulmonary Involvement ◽  
Multiple Organ ◽  
Langerhans Cell ◽  
Liver Involvement ◽  
Unknown Etiology ◽  
Multisystem Disease ◽  
Organ Systems

Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of Langerhans cells of unknown etiology. It can involve multiple organ systems with different clinical presentation, which complicates the diagnosis. It can range from isolated to multisystem disease with different prognosis. Although common among children, liver involvement is relatively rare in adults and frequently overlooked. Natural history of liver LCH fits into two stages: an early stage with infiltration by histiocytes and a late stage with sclerosis of the biliary tree. Pulmonary findings are more common and include multiple nodules in different stages of cavitation, predominantly in the upper lobes. We present a case of adult LCH with pulmonary and biopsy proven liver involvement with resolution of the hepatic findings after treatment.

scholarly journals Multisystemic langerhans-cell histiocytosis with dominant lesions of the lung parenchyma

2020 ◽  
Vol 23 (80) ◽  
pp. 26-28
Author(s):  
Jovan Javorac ◽  
Dejan Živanović ◽  
Miroslav Ilić ◽  
Ana Milenković
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Lung Parenchyma ◽  
Langerhans Cell ◽  
Myeloid Dendritic Cells ◽  
System Disease ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Initial Stage ◽  
Multisystemic Disease

Langerhans cell histiocytosis (LCH) is a rare disease of unknown aetiology characterized by abnormal monoclonal proliferation of CD1a+/ CD207+ myeloid dendritic cells (Langerhans cells) in various organs, including bones, skin, lymph nodes, liver, lungs, central nervous system (CNS). Pulmonary Langerhans cell histiocytosis (PLCH) may occur as single-system disease or the lungs may be affected within a multisystemic disease. This paper presents cases of two patients with multisystemic LCH, with predominant lung involvement. In the first patient, the disease is in the terminal stage, with chronic respiratory failure requiring long-term oxygen therapy at home and development of severe pulmonary hypertension, so the lung transplantation may be considered as the only potentially effective therapeutic procedure at this moment. The second patient is in the initial stage of the disease, with satisfactory pulmonary function for now, so it could be considered to use some new targeted therapy to prevent the progression of the disease. Thus, it is essential to consistently apply, as soon as possible, all available pharmacological treatments, as well as adequate palliative and health care, to ensure a decent quality of life for these patients.

scholarly journals Pulmonary Langerhans cell histiocytosis with Skin involvement; an amazing case report

2019 ◽  
Vol 7 (1) ◽  
pp. 32-36
Author(s):  
Sara Saniee ◽  
Niloofar Afshari ◽  
Saeed Kashefi
Keyword(s):  
Case Report ◽  
Langerhans Cell Histiocytosis ◽  
Pulmonary Involvement ◽  
Medical Problem ◽  
Langerhans Cell ◽  
Pathology Report ◽  
Skin Involvement ◽  
Recurrent Pneumothorax ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Progressive Dyspnea

Introduction: Langerhans cell histiocytosis (LCH) is a rare disease characterized by infiltration of large mononuclear or dendritic cells in one or more organs. Lung can be involved alone or with other organs in this complication. Case Report: We report a 33-year-old smoker man with LCH in whom both the lung and the skin were involved. He was referred to our department due to malodourous, crusted and scaly erythematous patches, plaques, and pustules involved scalp and flexural area. His medical problem started with pulmonary involvement. He experienced spontaneous pneumothorax, and was suffering progressive dyspnea for years. Cryo-transbronchial lung biopsy previously had been done. Pathology report was pulmonary LCH (PLCH). Weid skin biopsy. Histologic analysis of skin with haemotoxylin and eosin (H&E) staining and immunohistochemistry (IHC) exam with S100 and CDa1 confirmed the diagnosis of LCH. Conclusion: It is important to consider PLCH in the setting of recurrent pneumothorax and progressive dyspnea in middle-aged smoker patient. PLCH may be associated with skin involvement.

Long-term Outcomes of Adult Pulmonary Langerhans Cell Histiocytosis: A Prospective Cohort

2021 ◽  
pp. 2101017
Author(s):  
Amira Benattia ◽  
Emmanuelle Bugnet ◽  
Anouk Walter-Petrich ◽  
Constance de Margerie-Mellon ◽  
Véronique Meignin ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Pulmonary Involvement ◽  
Langerhans Cell ◽  
High Rate ◽  
Long Term Outcomes ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Reference Centre ◽  
Kaplan Meier ◽  
Extra Pulmonary

BackgroundThe long-term outcomes of adult pulmonary Langerhans cell histiocytosis (PLCH), particularly survival, are largely unknown. Two earlier retrospective studies reported a high rate of mortality, which contrasts with our clinical experience.MethodsTo address this issue, all newly diagnosed PLCH patients referred to the French national reference centre for histiocytoses between 2004 and 2018 were eligible for inclusion. The primary outcome was survival, which was defined as the time from inclusion to lung transplantation or death from any cause. Secondary outcomes included the cumulative incidences of chronic respiratory failure (CRF), pulmonary hypertension (PH), malignant diseases, and extra-pulmonary involvement in initially isolated PLCH. Survival was estimated using the Kaplan-Meier method.ResultsTwo hundred six patients (mean age: 39±13 years, 60% females, 95% current smokers) were prospectively followed for a median duration of 5.1 years (interquartile range [IQR], 3.2 to 7.6). Twelve (6%) patients died. The estimated rate of survival at 10 years was 93% (95% confidence interval [CI], 89–97). The cumulative incidences of CRF and/or PH were less than 5% at both 5 and 10 years, and 58% of these patients died. Twenty-seven malignancies were observed in 23 patients. The estimated standardized incidence ratio of lung carcinoma was 17.0 (95% CI, 7.45–38.7) compared to an age- and sex-matched French population. Eight (5.1%) of the 157 patients with isolated PLCH developed extra-pulmonary involvement.ConclusionsThe long-term prognosis of PLCH is significantly more favourable than was previously reported. Patients must be closely monitored after diagnosis to detect severe complications early.

Radiologic manifestation of pulmonary Langerhans' cell histiocytosis

1993 ◽  
Vol 29 (5) ◽  
pp. 973
Author(s):  
Jong Sung Kim ◽  
Duk Ja Bang ◽  
Hyun Chul Rhim ◽  
Seok Chol Jeon ◽  
Seung Ro Lee ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Pulmonary Langerhans Cell Histiocytosis
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