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Ueno M, Oku H, Todoroki Y, Murakami Y, Hayashida Y, Yamasaki K, Yatera K, Katafuchi E, Shimajiri S, Aoki T. A Case of Nonsmoker Pulmonary Langerhans Cell Histiocytosis With Multiple Pulmonary Nodules Disappeared and Appeared. J Thorac Imaging 2024; 39:W104-W107. [PMID: 39439377 DOI: 10.1097/rti.0000000000000810] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/25/2024]
Abstract
We present a non-smoker woman in her 40s with PLCH who presented with atypical imaging findings of multiple pulmonary noncavitary nodules without air cysts with repeated waxing and waning.
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Affiliation(s)
- Midori Ueno
- Department of Radiology, University of Occupational and Environmental Health, Japan
| | - Haruka Oku
- Department of Radiology, University of Occupational and Environmental Health, Japan
| | - Yo Todoroki
- Department of Radiology, University of Occupational and Environmental Health, Japan
| | - Yu Murakami
- Department of Radiology, University of Occupational and Environmental Health, Japan
| | - Yoshiko Hayashida
- Department of Radiology, University of Occupational and Environmental Health, Japan
| | - Kei Yamasaki
- Department of Respiratory Medicine, University of Occupational and Environmental Health, Japan
| | - Kazuhiro Yatera
- Department of Respiratory Medicine, University of Occupational and Environmental Health, Japan
| | - Eisuke Katafuchi
- Department of Pathology and Cell Biology, University of Occupational and Environmental Health, Japan
| | - Shohei Shimajiri
- Department of Pathology and Cell Biology, University of Occupational and Environmental Health, Japan
| | - Takatoshi Aoki
- Department of Radiology, University of Occupational and Environmental Health, Japan
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Ishimoto H, Sakamoto N, Ozasa M, Katoh T, Itonaga H, Wataya M, Takao D, Hara A, Kido T, Yamaguchi H, Yamamoto K, Obase Y, Ishimatsu Y, Miyazaki Y, Mukae H. Pulmonary Langerhans Cell Histiocytosis That Progressed from a Single-system to a Multisystem Form despite Smoking Cessation. Intern Med 2023; 62:877-880. [PMID: 35945010 PMCID: PMC10076126 DOI: 10.2169/internalmedicine.0139-22] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
Abstract
A 36-year-old Japanese man presented with cavities and nodular shadows in the lower lobes of his lungs and osteolytic lesions in the thoracic spine. He was diagnosed with multisystem Langerhans cell histiocytosis (LCH). Three years earlier, he had been noted to have small cavities and granular lesions noted in the upper lobes of his lungs, which later improved with smoking cessation. It was likely that his single-system pulmonary LCH (PLCH) progressed to multisystem LCH despite smoking cessation. Relapse or progression may occur in cases where PLCH lesions improve after smoking cessation. Thus, close follow-up is vital.
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Affiliation(s)
- Hiroshi Ishimoto
- Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
| | - Noriho Sakamoto
- Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
| | - Mutsumi Ozasa
- Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
- Department of Pathology, Nagasaki University Graduate School of Biomedical Sciences, Japan
| | - Takeharu Katoh
- Department of Hematology, Nagasaki University Hospital, Japan
| | | | - Makoto Wataya
- Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
- Medical Education Development Center, Nagasaki University Hospital, Japan
| | - Daisuke Takao
- Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
| | - Atsuko Hara
- Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
| | - Takashi Kido
- Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
| | - Hiroyuki Yamaguchi
- Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
| | - Kazuko Yamamoto
- Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
| | - Yasushi Obase
- Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
| | - Yuji Ishimatsu
- Department of Nursing, Nagasaki University Graduate School of Biomedical Sciences, Japan
| | - Yasushi Miyazaki
- Department of Hematology, Nagasaki University Hospital, Japan
- Department of Hematology, Atomic Bomb Disease and Hibakusha Medicine Unit, Atomic Bomb Disease Institute, Nagasaki University, Japan
| | - Hiroshi Mukae
- Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
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McClain KL, Bigenwald C, Collin M, Haroche J, Marsh RA, Merad M, Picarsic J, Ribeiro KB, Allen CE. Histiocytic disorders. Nat Rev Dis Primers 2021; 7:73. [PMID: 34620874 PMCID: PMC10031765 DOI: 10.1038/s41572-021-00307-9] [Citation(s) in RCA: 83] [Impact Index Per Article: 20.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 09/07/2021] [Indexed: 12/18/2022]
Abstract
The historic term 'histiocytosis' meaning 'tissue cell' is used as a unifying concept for diseases characterized by pathogenic myeloid cells that share histological features with macrophages or dendritic cells. These cells may arise from the embryonic yolk sac, fetal liver or postnatal bone marrow. Prior classification schemes align disease designation with terminal phenotype: for example, Langerhans cell histiocytosis (LCH) shares CD207+ antigen with physiological epidermal Langerhans cells. LCH, Erdheim-Chester disease (ECD), juvenile xanthogranuloma (JXG) and Rosai-Dorfman disease (RDD) are all characterized by pathological ERK activation driven by activating somatic mutations in MAPK pathway genes. The title of this Primer (Histiocytic disorders) was chosen to differentiate the above diseases from Langerhans cell sarcoma and malignant histiocytosis, which are hyperproliferative lesions typical of cancer. By comparison LCH, ECD, RDD and JXG share some features of malignant cells including activating MAPK pathway mutations, but are not hyperproliferative. 'Inflammatory myeloproliferative neoplasm' may be a more precise nomenclature. By contrast, haemophagocytic lymphohistiocytosis is associated with macrophage activation and extreme inflammation, and represents a syndrome of immune dysregulation. These diseases affect children and adults in varying proportions depending on which of the entities is involved.
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Affiliation(s)
- Kenneth L McClain
- Texas Children's Cancer Center, Department of Paediatrics, Baylor College of Medicine, Houston, TX, USA.
| | - Camille Bigenwald
- Department of Oncological Sciences and Translational and Molecular Imaging Institute, Icahn School of Medicine at Mount Sinai, New York, NY, USA
| | - Matthew Collin
- Human Dendritic Cell Lab, Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK
| | - Julien Haroche
- Department of Internal Medicine, Institut E3M French Reference Centre for Histiocytosis, Pitié-Salpȇtrière Hospital, Assistance Publique-Hôpitaux de Paris, Sorbonne Université, Paris, France
| | - Rebecca A Marsh
- Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital Medical Center, and University of Cincinnati, Cincinnati, OH, USA
| | - Miriam Merad
- Department of Oncological Sciences and Translational and Molecular Imaging Institute, Icahn School of Medicine at Mount Sinai, New York, NY, USA
| | - Jennifer Picarsic
- Division of Pathology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA
| | - Karina B Ribeiro
- Faculdade de Ciȇncias Médicas da Santa Casa de São Paulo, Department of Collective Health, São Paulo, Brazil
| | - Carl E Allen
- Texas Children's Cancer Center, Department of Paediatrics, Baylor College of Medicine, Houston, TX, USA
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Wang FF, Liu YS, Zhu WB, Liu YD, Chen Y. Pulmonary Langerhans cell histiocytosis in adults: A case report. World J Clin Cases 2019; 7:1892-1898. [PMID: 31417936 PMCID: PMC6692265 DOI: 10.12998/wjcc.v7.i14.1892] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/06/2019] [Revised: 06/16/2019] [Accepted: 06/21/2019] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Langerhans cell histiocytosis (LCH) is a rare disease of unknown aetiology. While it may affect any organ of the body, few cases of solitary lung involvement are published in the literature. Here, we report a rare case of pulmonary LCH (PLCH) in an adult.
CASE SUMMARY A 52-year-old male presented to hospital in July 2018 with complaints of progressively worsening cough with sputum, breathlessness, easy fatigability, and loss of appetite since 2016, and a 32-year history of heavy cigarette smoking (average 30 cigarettes/d). Physical examination showed only weakened breathing sounds and wheezing during lung auscultation. Chest computed tomography (CT) showed irregular micronodules and multiple thin-walled small holes. Respiratory function tests showed a slight decrease. Ultrasonic cardiogram showed mild tricuspid regurgitation and no pulmonary hypertension. Fibreoptic bronchoscopy was performed with transbronchial biopsies from the basal segment of right lower lobe. LCH was confirmed by immunohistochemistry. The final diagnosis was PLCH without extra-pulmonary involvement. We suggested smoking cessation treatment. A 3-mo follow-up chest CT scan showed clear absorption of the nodule and thin-walled small holes. The symptoms of cough and phlegm had improved markedly and appetite had improved. There was no obvious dyspnoea.
CONCLUSION Imaging manifestations of nodules, cavitating nodules, and thick-walled or thin-walled cysts prompted suspicion of PLCH and lung biopsy for diagnosis.
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Affiliation(s)
- Feng-Feng Wang
- Department of Respiration, People’s Hospital of Jingjiang, YangZhou University Medical Academy, Jingjiang 214500, Jiangsu Province, China
| | - Ya-Shuang Liu
- Department of Respiration, People’s Hospital of Jingjiang, YangZhou University Medical Academy, Jingjiang 214500, Jiangsu Province, China
| | - Wei-Bo Zhu
- Department of Respiration, People’s Hospital of Jingjiang, YangZhou University Medical Academy, Jingjiang 214500, Jiangsu Province, China
| | - Yan-Dong Liu
- Department of Respiration, People’s Hospital of Jingjiang, YangZhou University Medical Academy, Jingjiang 214500, Jiangsu Province, China
| | - Yao Chen
- Department of Respiration, People’s Hospital of Jingjiang, YangZhou University Medical Academy, Jingjiang 214500, Jiangsu Province, China
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Pneumothorax in Patients with Pulmonary Langerhans Cell Histiocytosis. Lung 2018; 196:715-720. [PMID: 30187131 PMCID: PMC6244644 DOI: 10.1007/s00408-018-0155-1] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/05/2018] [Accepted: 08/28/2018] [Indexed: 11/19/2022]
Abstract
Introduction Pneumothorax often develops in pulmonary Langerhans cell histiocytosis (PLCH), but some patients take a long time to be correctly diagnosed. Objectives This study assessed the frequency of pneumothorax in PLCH and analysed the role of chest computed tomography (CT) in the prompt diagnosis. Patients and material Of the 90 patients with PLCH seen from 2000 to 2015, 29 (32%) had pneumothorax as the initial finding. In this group, 18 (62%) patients were diagnosed within 1 month, whereas the diagnosis was delayed for 4–120 months in 11 (38%) patients. Results Patients who had pneumothorax as the initial sign of PLCH tended to be younger (mean age 27.7 ± 7.92 vs. 39.9 ± 13.21 years; P = 0.0001), male (69% vs. 43%; P = 0.028), smoked less (mean pack/years 8.4 ± 6.85 vs. 19 ± 17.16; P = 0.003), and had a significantly lower mean FVC (77.96 ± 19.62 vs. 89.47 ± 21.86% pred.; P = 0.015) and FEV1 (68.6 ± 19.93 vs. 79.4 ± 21.48% pred.; P = 0.03 than patients who had no pneumothorax. Recurrent pneumothorax was diagnosed more frequently in the group with a delayed diagnosis (82% vs. 39%; P = 0.02). CT was performed in all of the patients who were diagnosed promptly, but in none of the patients with a delayed diagnosis. Conclusions Patients who had pneumothorax as the initial sign of PLCH were younger, more frequently men, and had greater respiratory impairment than those who had no pneumothorax. CT in patients with pneumothorax led to a correct diagnosis of this disease.
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Lorillon G, Tazi A. How I manage pulmonary Langerhans cell histiocytosis. Eur Respir Rev 2017; 26:26/145/170070. [PMID: 28877978 DOI: 10.1183/16000617.0070-2017] [Citation(s) in RCA: 29] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/15/2017] [Accepted: 07/12/2017] [Indexed: 02/07/2023] Open
Abstract
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare sporadic cystic lung disease of unknown aetiology that is characterised by the infiltration and destruction of the wall of distal bronchioles by CD1a+ Langerhans-like cells. In adults, PLCH is frequently isolated and affects young smokers of both sexes. Recent multicentre studies have led to the more standardised management of patients in clinical practice. Smoking cessation is essential and is occasionally the only suitable intervention. Serial lung function testing is important because a significant proportion of patients may experience an early decline in forced expiratory volume in 1 s and develop airflow obstruction. Cladribine was reported to dramatically improve progressive PLCH in some patients. Its efficacy and tolerance are currently being evaluated. Patients who complain of unexplained dyspnoea with decreased diffusing capacity of the lung for carbon monoxide should be screened for pulmonary hypertension by Doppler echocardiography, which must be confirmed by right heart catheterisation. Lung transplantation is a therapeutic option for patients with advanced PLCH.The identification of the BRAFV600E mutation in approximately half of Langerhans cell histiocytosis lesions, including PLCH, and other mutations of the mitogen-activated protein kinase (MAPK) pathway in a subset of lesions has led to targeted treatments (BRAF and MEK (MAPK kinase) inhibitors). These treatments need to be rigorously evaluated because of their potentially severe side-effects.
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Affiliation(s)
- Gwenaël Lorillon
- National Reference Centre for Histiocytoses, Pulmonary Dept, Assistance Publique-Hôpitaux de Paris, Hôpital Saint-Louis, Paris, France
| | - Abdellatif Tazi
- National Reference Centre for Histiocytoses, Pulmonary Dept, Assistance Publique-Hôpitaux de Paris, Hôpital Saint-Louis, Paris, France .,University Paris Diderot, Sorbonne, Paris Cité, Inserm UMR-1153 (CRESS), Biostatistics and Clinical Epidemiology Research Team (ECSTRA), Paris, France
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Lung Function in Pregnancy in Langerhans Cell Histiocytosis. ADVANCES IN EXPERIMENTAL MEDICINE AND BIOLOGY 2017; 1023:73-83. [PMID: 28744781 DOI: 10.1007/5584_2017_72] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/06/2023]
Abstract
Pulmonary Langerhans cell histiocytosis (LCH) is a rare disease, affecting usually young people. The course of the disease is variable. In some pulmonary LCH patients a severe lung destruction and progression in spite of chemotherapy is observed, but in others just a cessation of smoking induces a regression of the disease. In the present study we seek to determine the influence of pregnancy on pulmonary function in LCH patients, an unchartered area of research. We addressed the issue by investigating eight pregnant women out of the 45 women hospitalized with the diagnosis of pulmonary LCH in the period from 2000 to 2015. For five of the eight pregnant women it was the second gestation. The median follow-up period was 120 months (range 72-175 months). Ten healthy children were born by a C-section. Two spontaneous miscarriages in the seventh week of gestation, and one tubal ectopic pregnancy were recorded. We found that pregnancy did not significantly influence pulmonary function assessed by the following indices: forced expiratory volume in 1 s (FEV1), lung vital capacity (VC), total lung capacity (TLC), residual volume (RV), diffusing capacity of the lungs for carbon monoxide (DLCO), and the distance and arterial oxygen saturation in 6-min walk test. Only one patient in the third trimester of pregnancy experienced bilateral pneumothorax, with persistent air leak. In all patients, delivery and postpartum period were uneventful. We conclude that pregnancy in pulmonary LCH patients is safe and not associated with deterioration of pulmonary function or blood oxygenation.
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Abstract
PURPOSE OF REVIEW To discuss the imaging of interstitial lung disease believed to be caused by smoking. RECENT FINDINGS It is increasingly clear that smoking is associated with a variety of patterns of interstitial lung disease. The radiologic features of interstitial lung disease caused by smoking cigarettes are variable and may be nonspecific. SUMMARY It is now accepted that cigarette smoking can cause lung diseases other than lung cancer, chronic bronchitis and emphysema. Indeed, the hypothesis that tobacco smoke can cause interstitial lung disease - and, specifically, pulmonary fibrosis - dates back to the 1960s. The list of interstitial lung disease, in which smoking is believed to have an etiologic role, includes Langerhans' cell histiocytosis, respiratory bronchiolitis/respiratory bronchiolitis-interstitial lung disease and desquamative interstitial pneumonia. More recently, there is emerging evidence which suggests that smoking may be associated with other patterns of pulmonary fibrosis (e.g. nonspecific interstitial pneumonia and smoking-related interstitial fibrosis). In the present review we discuss the imaging of the interstitial lung disease known to be caused by smoking; the typical appearances and some of the diagnostic difficulties are discussed.
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Ferreira Francisco FA, Soares Souza A, Zanetti G, Marchiori E. Multiple cystic lung disease. Eur Respir Rev 2015; 24:552-64. [DOI: 10.1183/16000617.0046-2015] [Citation(s) in RCA: 41] [Impact Index Per Article: 4.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022] Open
Abstract
Multiple cystic lung disease represents a diverse group of uncommon disorders that can present a diagnostic challenge due to the increasing number of diseases associated with this presentation. High-resolution computed tomography of the chest helps to define the morphological aspects and distribution of lung cysts, as well as associated findings. The combination of appearance upon imaging and clinical features, together with extrapulmonary manifestations, when present, permits confident and accurate diagnosis of the majority of these diseases without recourse to open-lung biopsy. The main diseases in this group that are discussed in this review are lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis and folliculin gene-associated syndrome (Birt–Hogg–Dubé); other rare causes of cystic lung disease, including cystic metastasis of sarcoma, are also discussed. Disease progression is unpredictable, and understanding of the complications of cystic lung disease and their appearance during evolution of the disease are essential for management. Correlation of disease evolution and clinical context with chest imaging findings provides important clues for defining the underlying nature of cystic lung disease, and guides diagnostic evaluation and management.
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Tazi A, de Margerie C, Naccache JM, Fry S, Dominique S, Jouneau S, Lorillon G, Bugnet E, Chiron R, Wallaert B, Valeyre D, Chevret S. The natural history of adult pulmonary Langerhans cell histiocytosis: a prospective multicentre study. Orphanet J Rare Dis 2015; 10:30. [PMID: 25887097 PMCID: PMC4438520 DOI: 10.1186/s13023-015-0249-2] [Citation(s) in RCA: 65] [Impact Index Per Article: 6.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/10/2014] [Accepted: 03/03/2015] [Indexed: 12/27/2022] Open
Abstract
BACKGROUND The natural history of pulmonary Langerhans cell histiocytosis (PLCH) has been unclear due to the absence of prospective studies. The rate of patients who experience an early progression of their disease is unknown. Additionally, conflicting effects of smoking cessation on the outcome of PLCH have been reported. METHODS In this prospective, multicentre study, 58 consecutive patients with newly diagnosed PLCH were comprehensively evaluated over a two-year period. Our objectives were to estimate the incidence of early progression of the disease and to evaluate the impact of smoking status on lung function outcomes. Lung function deterioration was defined as a decrease of at least 15% in FEV1 and/or FVC and/or DLCO, compared with baseline values. At each visit, smoking status was recorded based on the patients' self-reports and urinary cotinine measurements that were blinded for the patients. The cumulative incidence of lung function outcomes over time was estimated using the non-parametric Kaplan-Meier method. Multivariate Cox models with time-dependent covariates were used to calculate the hazards ratios of the lung function deterioration associated with smoking status with adjustment for potential confounders. RESULTS The cumulative incidence of lung function deterioration at 24 months was 38% (22% for FEV1 and DLCO, and 9% for FVC). In the multivariate analysis, smoking status and PaO2 at inclusion were the only factors associated with the risk of lung function deterioration. The patients' smoking statuses markedly changed over time. Only 20% of the patients quit using tobacco for the entire study period. Nevertheless, being a non-smoker was associated with a decreased risk of subsequent lung function deterioration, even after adjustment for baseline predictive factors. By serial lung computed tomography, the extent of cystic lesions increased in only 11% of patients. CONCLUSIONS Serial lung function evaluation on a three- to six-month basis is essential for the follow-up of patients with recently diagnosed PLCH to identify those who experience an early progression of their disease. These patients are highly addicted to tobacco, and robust efforts should be undertaken to include them in smoking cessation programs. TRIAL REGISTRATION ClinicalTrials.gov: No: NCT01225601 .
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Affiliation(s)
- Abdellatif Tazi
- Assistance Publique-Hôpitaux de Paris, Hôpital Saint-Louis, Centre National de Référence de l'histiocytose Langerhansienne, Service de Pneumologie, 1 Avenue Claude Vellefaux, 75475, Paris Cedex 10, France. .,Univ Paris Diderot, Sorbonne Paris Cité, U1153 CRESS, Biostatistics and Clinical Epidemiology research team, Paris, France.
| | - Constance de Margerie
- Assistance Publique-Hôpitaux de Paris, Service de Radiologie, Hôpital Saint-Louis, Paris, France.
| | - Jean Marc Naccache
- Assistance Publique-Hôpitaux de Paris, Service de Pneumologie, Hôpital Avicenne, Bobigny, France.
| | - Stéphanie Fry
- Service de Pneumologie et Immuno-allergologie, Centre de compétence des maladies pulmonaires rares, Hôpital Calmette, Lille, France.
| | | | - Stéphane Jouneau
- IRSET UMR 1085, Université de Rennes 1; Service de Pneumologie, Hôpital Pontchaillou, Rennes, France.
| | - Gwenaël Lorillon
- Assistance Publique-Hôpitaux de Paris, Hôpital Saint-Louis, Centre National de Référence de l'histiocytose Langerhansienne, Service de Pneumologie, 1 Avenue Claude Vellefaux, 75475, Paris Cedex 10, France.
| | - Emmanuelle Bugnet
- Assistance Publique-Hôpitaux de Paris, Hôpital Saint-Louis, Centre National de Référence de l'histiocytose Langerhansienne, Service de Pneumologie, 1 Avenue Claude Vellefaux, 75475, Paris Cedex 10, France.
| | - Raphael Chiron
- Département de Pneumologie, Hôpital Arnaud de Villeneuve, Montpellier, France.
| | - Benoit Wallaert
- Service de Pneumologie et Immuno-allergologie, Centre de compétence des maladies pulmonaires rares, Hôpital Calmette, Lille, France. .,Université Lille 2, Lille, France.
| | - Dominique Valeyre
- Université Paris 13, Sorbonne Paris Cité; Assistance Publique-Hôpitaux de Paris, Service de Pneumologie, Hôpital Avicenne, Bobigny, France.
| | - Sylvie Chevret
- Univ Paris Diderot, Sorbonne Paris Cité, U1153 CRESS, Biostatistics and Clinical Epidemiology research team, Paris, France. .,Assistance Publique-Hôpitaux de Paris; Hôpital Saint-Louis, Service de Biostatistique et Information Médicale, Paris, France.
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Grobost V, Khouatra C, Lazor R, Cordier JF, Cottin V. Effectiveness of cladribine therapy in patients with pulmonary Langerhans cell histiocytosis. Orphanet J Rare Dis 2014; 9:191. [PMID: 25433492 PMCID: PMC4268858 DOI: 10.1186/s13023-014-0191-8] [Citation(s) in RCA: 47] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/02/2014] [Accepted: 11/11/2014] [Indexed: 12/27/2022] Open
Abstract
Background Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disorder characterised by granulomatous proliferation of CD1a-positive histiocytes forming granulomas within lung parenchyma, in strong association with tobacco smoking, and which may result in chronic respiratory failure. Smoking cessation is considered to be critical in management, but has variable effects on outcome. No drug therapy has been validated. Cladribine (chlorodeoxyadenosine, 2-CDA) down-regulates histiocyte proliferation and has been successful in curbing multi-system Langerhans cell histiocytosis and isolated PLCH. Methods and patients We retrospectively studied 5 patients (aged 37–55 years, 3 females) with PLCH who received 3 to 4 courses of cladribine therapy as a single agent (0.1 mg/kg per day for 5 consecutive days at monthly intervals). One patient was treated twice because of relapse at 1 year. Progressive pulmonary disease with obstructive ventilatory pattern despite smoking cessation and/or corticosteroid therapy were indications for treatment. Patients were administered oral trimethoprim/sulfamethoxazole and valaciclovir to prevent opportunistic infections. They gave written consent to receive off-label cladribine in the absence of validated treatment. Results Functional class dyspnea improved with cladribine therapy in 4 out of 5 cases, and forced expiratory volume in 1 second (FEV1) increased in all cases by a mean of 387 ml (100–920 ml), contrasting with a steady decline prior to treatment. Chest high-resolution computed tomography (HRCT) features improved with cladribine therapy in 4 patients. Hemodynamic improvement was observed in 1 patient with pre-capillary pulmonary hypertension. The results suggested a greater treatment effect in subjects with nodular lung lesions and/or thick-walled cysts on chest HRCT, with diffuse hypermetabolism of lung lesions on positron emission tomography (PET)-scan, and with progressive disease despite smoking cessation. Infectious pneumonia developed in 1 patient, with later grade 4 neutrocytopenia but without infection. Discussion Data interpretation was limited by the retrospective, uncontrolled study design and small sample size. Conclusion Cladribine as a single agent may be effective therapy in patients with progressive PLCH.
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Affiliation(s)
- Vincent Grobost
- National Reference Centre for Rare Pulmonary Diseases, Department of Respiratory Medicine, Louis Pradel Hospital; Claude Bernard Lyon 1 University, Lyon, UMR 754, France.
| | - Chahera Khouatra
- National Reference Centre for Rare Pulmonary Diseases, Department of Respiratory Medicine, Louis Pradel Hospital; Claude Bernard Lyon 1 University, Lyon, UMR 754, France.
| | - Romain Lazor
- National Reference Centre for Rare Pulmonary Diseases, Department of Respiratory Medicine, Louis Pradel Hospital; Claude Bernard Lyon 1 University, Lyon, UMR 754, France. .,Department of Medicine, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.
| | - Jean-François Cordier
- National Reference Centre for Rare Pulmonary Diseases, Department of Respiratory Medicine, Louis Pradel Hospital; Claude Bernard Lyon 1 University, Lyon, UMR 754, France.
| | - Vincent Cottin
- National Reference Centre for Rare Pulmonary Diseases, Department of Respiratory Medicine, Louis Pradel Hospital; Claude Bernard Lyon 1 University, Lyon, UMR 754, France. .,Hospices Civils de Lyon, Hôpital Louis Pradel, Centre national de référence des maladies pulmonaires rares, Centre de compétences de l'hypertension pulmonaire, Service de pneumologie, Université de Lyon, Université Claude Bernard Lyon 1, INRA, Lyon, UMR754, France.
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Castoldi MC, Verrioli A, De Juli E, Vanzulli A. Pulmonary Langerhans cell histiocytosis: the many faces of presentation at initial CT scan. Insights Imaging 2014; 5:483-92. [PMID: 24996395 PMCID: PMC4141336 DOI: 10.1007/s13244-014-0338-0] [Citation(s) in RCA: 30] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/06/2014] [Revised: 05/12/2014] [Accepted: 05/19/2014] [Indexed: 12/22/2022] Open
Abstract
Objectives 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. Methods 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. Results 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 lymphangiomyomatosis. Clinical symptoms are usually non-specific, although a history of cigarette smoking, coupled with the presence of typical or suggestive findings at imaging, is key to suspecting the disease. Atypical presentations require surgical biopsy for diagnosis. Conclusions The radiologist should be familiar with PLCH imaging features to correctly diagnose the disease or need for further investigation. Teaching Points • PLCH is a rare interstitial smoking-related disease that usually affects young adults. • The typical first CT shows a mix of nodules, cavitary nodules and cysts in the upper-middle lungs. • Atypical appearance, either cysts or nodules alone, mandates that other diagnoses be considered. • Lung cystic involvement correlates with lung function abnormalities and predicts functional decline. • Integration of the clinical history and imaging results is key to diagnosis.
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Affiliation(s)
- M C Castoldi
- Department of Radiology, Ospedale CTO, via Bignami 1, 20162, Milan, Italy,
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Suri HS, Yi ES, Nowakowski GS, Vassallo R. Pulmonary langerhans cell histiocytosis. Orphanet J Rare Dis 2012; 7:16. [PMID: 22429393 PMCID: PMC3342091 DOI: 10.1186/1750-1172-7-16] [Citation(s) in RCA: 94] [Impact Index Per Article: 7.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/16/2011] [Accepted: 03/19/2012] [Indexed: 12/25/2022] Open
Abstract
Pulmonary Langerhans Cell Histiocytosis (PLCH) is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. The pathologic hallmark of PLCH is the accumulation of Langerhans and other inflammatory cells in small airways, resulting in the formation of nodular inflammatory lesions. While the overwhelming majority of patients are smokers, mechanisms by which smoking induces this disease are not known, but likely involve a combination of events resulting in enhanced recruitment and activation of Langerhans cells in small airways. Bronchiolar inflammation may be accompanied by variable lung interstitial and vascular involvement. While cellular inflammation is prominent in early disease, more advanced stages are characterized by cystic lung destruction, cicatricial scarring of airways, and pulmonary vascular remodeling. Pulmonary function is frequently abnormal at presentation. Imaging of the chest with high resolution chest CT scanning may show characteristic nodular and cystic abnormalities. Lung biopsy is necessary for a definitive diagnosis, although may not be required in instances were imaging findings are highly characteristic. There is no general consensus regarding the role of immunosuppressive therapy in smokers with PLCH. All smokers must be counseled on the importance of smoking cessation, which may result in regression of disease and obviate the need for systemic immunosuppressive therapy. The prognosis for most patients is relatively good, particularly if longitudinal lung function testing shows stability. Complications like pneumothoraces and secondary pulmonary hypertension may shorten life expectancy. Patients with progressive disease may require lung transplantation.
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Affiliation(s)
- Harpreet S Suri
- Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA
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15
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Abstract
Pulmonary Langerhans' cell histiocytosis (PLCH) is an unusual cystic lung disease that is also characterized by extrapulmonary manifestations. The current review discusses the presenting features and relevant diagnostic testing and treatment options for PLCH in the context of a clinical case. While the focus of the present article is adult PLCH and its pulmonary manifestations, it is important for clinicians to distinguish the adult and pediatric forms of the disease, as well as to be alert for possible extrapulmonary complications. A major theme of the current series of articles on rare lung diseases has been the translation of insights gained from fundamental research to the clinic. Accordingly, the understanding of dendritic cell biology in this disease has led to important advances in the care of patients with PLCH.
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Feuillet S, Giroux-Leprieur B, Tazi A. [Pulmonary Langerhans cell histiocytosis in adults]. Presse Med 2009; 39:107-15. [PMID: 19959324 DOI: 10.1016/j.lpm.2009.10.008] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/27/2009] [Accepted: 10/28/2009] [Indexed: 11/19/2022] Open
Abstract
Pulmonary Langerhans-cell histiocytosis in adults is a rare condition of unknown etiology characterized by the accumulation of Langerhans cells organized in granulomas involving the distal bronchioles and destroying their walls. It occurs in young subjects who smoke, with frequency peaking between 20 and 40 years. High-resolution thoracic CT is essential for diagnosis; in typical forms it shows a combination of nodules, cavitary nodules, thick-walled cysts, and thin-walled cysts. Diagnostic certainty requires a surgical lung biopsy, by videothoracoscopy, but only if a specialist considers it indicated. It is difficult to predict the disease course for any given patient. A prospective multicenter cohort study currently underway should provide more information about the natural history of this disease. Management is empirical, for efficacy has not been proved for any treatment. Stopping smoking is especially important to prevent the added development of chronic obstructive pulmonary disease (COPD), cardiovascular complications, or the onset of bronchopulmonary cancer, the frequency of which appears elevated in these patients. Oral corticosteroids are used to treat disease progression, especially in the symptomatic mainly nodular forms, but their efficacy for respiratory function has not been shown. Vinblastine, the reference treatment for multisystem forms of Langerhans-cell histiocytosis, is not indicated for pulmonary involvement in adults. Better knowledge of the pathogenic mechanisms involved in this condition should eventually make it possible to develop innovative treatment strategies. The creation of the national reference center for Langerhans-cell histiocytosis has given new momentum to clinical and pathophysiologic research on this orphan disease.
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Affiliation(s)
- Séverine Feuillet
- Centre de référence de l'histiocytose langerhansienne, Service de pneumologie, Hôpital Saint-Louis, F-75475 Paris Cedex 10, France
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Krajicek BJ, Ryu JH, Hartman TE, Lowe VJ, Vassallo R. Abnormal Fluorodeoxyglucose PET in Pulmonary Langerhans Cell Histiocytosis. Chest 2009; 135:1542-1549. [DOI: 10.1378/chest.08-1899] [Citation(s) in RCA: 42] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/01/2022] Open
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Niksarlioglu YO, Gurel B, Tezel GG, Firat P, Dogan R, Kars A, Coplu L. Langerhans' cell histiocytosis mimicking metastatic carcinoma of the lung. Respirology 2009; 14:456-8. [PMID: 19192223 DOI: 10.1111/j.1440-1843.2008.01474.x] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/11/2023]
Abstract
Langerhans' cell histiocytosis (LCH), previously known as Histiocytosis X, is characterized by abnormal accumulations of large mononuclear cells forming granulomas in various organs, mainly bone, skin and lung. This case report describes a 50-year-old man with a history of left pneumonectomy due to squamous cell carcinoma (SCC). During routine follow up, a CXR showed new parenchymal nodules in the right lung 57 months post treatment. Chest CT scan confirmed the presence of multiple parenchymal nodules. Open lung biopsy from the right upper lobe was performed and LCH was diagnosed. Re-analysis of the initial pneumonectomy specimen revealed no evidence of LCH in the surrounding lung tissue. On diagnosis, the patient stopped smoking and was treated with vinblastine and prednisolone for LCH. The nodules disappeared and have not returned in a further 18 months of follow up. In this patient LCH was diagnosed after SCC, which highlights that smoking-related diseases can be seen concomitantly or sequentially in the same patient.
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Affiliation(s)
- Yelda O Niksarlioglu
- Department of Chest Diseases, Hacettepe University Faculty of Medicine, Ankara, Turkey.
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19
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Negrin-Dastis S, Butenda D, Dorzee J, Fastrez J, d’Odémont JP. Complete disappearance of lung abnormalities on high-resolution computed tomography: a case of histiocytosis X. Can Respir J 2007; 14:235-7. [PMID: 17551600 PMCID: PMC2676369 DOI: 10.1155/2007/941618] [Citation(s) in RCA: 17] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/18/2022] Open
Abstract
A case of pulmonary Langerhans cell histiocytosis, proved by both lung high-resolution computed tomography and lung biopsy, is described. Following smoking cessation, lung nodules and cysts gradually disappeared on serial computed tomography scans, with complete clearance of the lesions after 12 months. The role of tobacco smoking is discussed, in detail, against the background of the literature.
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Affiliation(s)
| | | | - Jacques Dorzee
- Department of Radiology, CHR St-Joseph-Warquignies, Belgium
| | - Jacques Fastrez
- Department of Thoracic Surgery, CHR St-Joseph-Warquignies, Belgium
| | - Jean-Paul d’Odémont
- Department of Pneumology, CHR St-Joseph-Warquignies, Belgium
- Correspondence and reprints: Dr Jean-Paul d’Odémont, Department of Pneumology, CHR St-Joseph-Warquignies, 5-Avenue Baudouin de Constantinople, 7000 Mons, Belgium. Telephone 065-359177, fax 065-359060, e-mail
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20
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Sharma R, Maplethorpe R, Wilson G. Effect of pregnancy on lung function in adult pulmonary Langerhans cell histiocytosis. J Matern Fetal Neonatal Med 2006; 19:67-8. [PMID: 16492595 DOI: 10.1080/14767050500333835] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/25/2022]
Abstract
Adult pulmonary Langerhans cell histiocytosis (APLCH) is a rare lung disease. We report a case of APLCH and pregnancy with detailed lung function tests. There was no significant change in the lung function tests during pregnancy. It is apparent that pregnancy does not influence the evolution of pulmonary Langerhans cell histiocytosis.
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Affiliation(s)
- Rekha Sharma
- Department of Obstetrics & Gynecology, Newham University Hospital NHS Trust, London, UK.
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21
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Marten K, Hansell DM. Imaging of macrophage-related lung diseases. Eur Radiol 2005; 15:727-41. [PMID: 15633061 DOI: 10.1007/s00330-004-2554-3] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/13/2004] [Revised: 10/12/2004] [Accepted: 10/18/2004] [Indexed: 01/28/2023]
Abstract
Macrophage-related pulmonary diseases are a heterogeneous group of disorders characterized by macrophage accumulation, activation or dysfunction. These conditions include smoking-related interstitial lung diseases, metabolic disorders such as Niemann-Pick or Gaucher disease, and rare primary lung tumors. High-resolution computed tomography abnormalities include pulmonary ground-glass opacification secondary to infiltration by macrophages, centrilobular nodules or interlobular septal thickening reflecting peribronchiolar or septal macrophage accumulation, respectively, emphysema caused by macrophage dysfunction, and honeycombing following macrophage-related lung matrix remodeling.
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Affiliation(s)
- Katharina Marten
- Department of Radiology, Royal Brompton Hospital, Sydney Street, London, SW3 6NP, UK
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22
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Braier J, Latella A, Balancini B, Castaños C, Rosso D, Chantada G, Ripoli M, Goldberg J. Outcome in children with pulmonary Langerhans cell Histiocytosis. Pediatr Blood Cancer 2004; 43:765-9. [PMID: 15390304 DOI: 10.1002/pbc.20112] [Citation(s) in RCA: 58] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/09/2022]
Abstract
BACKGROUND The aim of this study was to evaluate features and outcome of children with Langerhans cell Histiocytosis (LCH) and pulmonary involvement. PROCEDURE Retrospective evaluation of LCH patients was performed from 1987 to 2001. Multisystem patients were classified according to the pattern of organ system involvement into Groups: A (no pulmonary, hematologic, or hepatic involvement), B (pulmonary involvement), C (pulmonary and hematologic or hepatic involvement), and D (hematologic or hepatic involvement). All had skin, bone, or lymph node involvement. Chest X-ray was performed in all patients and computed tomography (CT) in 21. Diagnostic lung biopsy was performed in five patients. RESULTS Pulmonary involvement was found in 36/220 patients studied. Two patients had isolated pulmonary involvement. Multisystem involvement was present in 83 patients, 34 of whom had pulmonary involvement. In 20/36 patients, tachypnea, cough, and thoracic pain occurred. Diffuse interstitial involvement was found in all cases. Pulmonary function tests were performed in nine patients, six of whom revealed mild to moderate restrictive respiratory involvement. The two patients having isolated pulmonary involvement survived 2 and 2.7 years after diagnosis. Median follow-up of all multisystem patients was 2.1 years, with a 5-year survival probability of 0.59. The 5-year survival probability of Groups A-D was 0.94, 0.83, 0.23, and 0.40, respectively. The survival difference between Groups B and C was statistically different (P < 0.0071). CONCLUSIONS According to our data, pulmonary compromise without other risk organ involvement does not appear to be a negative prognostic factor in our study.
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Affiliation(s)
- Jorge Braier
- Department of Hematology/Oncology, Hospital Nacional de Pediatría Juan P Garrahan, Buenos Aires, Argentina.
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23
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Flaherty KR, Martinez FJ. Cigarette smoking in interstitial lung disease: concepts for the internist. Med Clin North Am 2004; 88:1643-53, xiii. [PMID: 15464118 DOI: 10.1016/j.mcna.2004.08.001] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
Cigarette smoking is a common cause of lung disease. It is clearly implicated in the development of chronic obstructive pulmonary disease and lung cancer. Importantly, cigarette smoking has also been implicated in the development of interstitial lung diseases such as respiratory bronchiolitis interstitial lung disease, desquamative interstitial pneumonia, pulmonary Langerhans' cell histiocytosis,and idiopathic pulmonary fibrosis. The exact role of cigarette smoking in the development of interstitial lung diseases is still being defined; the relatively low prevalence of interstitial lung disease makes epidemiologic studies difficult.
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Affiliation(s)
- Kevin R Flaherty
- Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Michigan Health System, 1500 East Medical Center Drive, 3916 Taubman Center, Ann Arbor, MI 48109-0360, USA.
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25
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Abbott GF, Rosado-de-Christenson ML, Franks TJ, Frazier AA, Galvin JR. From the Archives of the AFIP. Radiographics 2004; 24:821-41. [PMID: 15143231 DOI: 10.1148/rg.243045005] [Citation(s) in RCA: 96] [Impact Index Per Article: 4.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Abstract
Pulmonary Langerhans cell histiocytosis (PLCH) is an isolated form of Langerhans cell histiocytosis that primarily affects cigarette smokers. PLCH is characterized by peribronchiolar proliferation of Langerhans cell infiltrates that form stellate nodules. The nodular lesions frequently cavitate and form thick- and thin-walled cysts, which are thought to represent enlarged airway lumina. PLCH lesions display temporal microscopic heterogeneity, with progression from dense cellular nodules to apparently cavitary nodules to increasing degrees of fibrosis that may extend along alveolar walls. In advanced cases, fibrotic scars are surrounded by enlarged, distorted air spaces. Affected patients are typically young adults who often present with cough and dyspnea. The characteristic radiographic features of PLCH are bilateral nodular and reticulonodular areas of opacity that predominantly involve the upper and middle lung zones with relative sparing of the lung bases. High-resolution computed tomography (CT) shows nodules and cysts in the same distribution and allows a confident prospective diagnosis of PLCH in the appropriate clinical setting. In typical cases, a predominantly nodular pattern is seen on CT scans in early phases of the disease, whereas a cystic pattern predominates in later phases. The radiologic abnormalities may regress, resolve completely, become stable, or progress to advanced cystic changes. Treatment consists of smoking cessation, but corticosteroid therapy may be useful in selected patients. Chemotherapeutic agents and lung transplantation may be offered to patients with advanced disease. The prognosis of PLCH is variable with frequent regression, stabilization, or recurrence of disease that does not correlate with cessation or continuation of smoking.
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Affiliation(s)
- Gerald F Abbott
- Department of Diagnostic Imaging, Brown Medical School, Rhode Island Hospital, 593 Eddy St, Providence, RI 02903, USA.
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26
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Vidal Serrano S, Rodríguez Becerra E, Marcilla Plaza D. Histiocitosis de células de Langerhans de larga evolución. Arch Bronconeumol 2004. [DOI: 10.1016/s0300-2896(04)75564-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/27/2022]
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Abstract
INTRODUCTION Adult pulmonary Langerhans'cell histiocytosis, also referred to as histiocytosis X, is a disorder of unknown etiology which affects preferentially young smokers. The disease is characterized by granulomatous lesions which progressively invade and destroy distal airways, leading to the formation of characteristic cicatricial kystic lesions. Florid granulomas contain numerous Langerhans'cells, antigen-presenting cells of the dendritic cell lineage, associated with T lymphocytes and eosinophils. The diagnosis rests on the combination of clinical and radiologic data, and particularly on high-resolution CT scan findings showing a typical association of nodular and cystic changes, predominantly in the upper and middle lobes. Further evaluation with surgical lung biopsy is indicated in less typical situations. CURRENT KNOWLEDGE AND KEY POINTS The pathogenesis of Langerhans'cell histiocytosis is not fully understood, but several arguments suggest that the disease results from an abnormal immune reaction initiated by Langerhans'cells and directed against the bronchial epithelium. Other arguments suggest the presence of genetic abnormalities susceptible, for example, to increase the sensitivity of these cells to cytokines (GM-CSF, or others) known to influence their survival and maturation. FUTURE PROSPECTS AND PROJECTS These recent advances in the pathogenesis of Langerhans'cell histiocytosis could promote the development of new therapeutic strategies designed to regulate the number and activated state of Langerhans'cells in specific lesions.
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Affiliation(s)
- P Soler
- Inserm U408, faculté Xavier-Bichat, BP 416, 75870 Paris cedex 18, France.
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28
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Desai SR, Ryan SM, Colby TV. Smoking-related interstitial lung diseases: histopathological and imaging perspectives. Clin Radiol 2003; 58:259-68. [PMID: 12662946 DOI: 10.1016/s0009-9260(02)00525-1] [Citation(s) in RCA: 39] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/15/2023]
Abstract
The present review focuses on the interstitial lung diseases related to smoking. Thus, the pathology and radiology of Langerhans cell histiocytosis, desquamative interstitial pneumonia, respiratory bronchiolitis and respiratory bronchiolitis-associated-interstitial lung disease are considered. The more tenuous association between pulmonary fibrosis and smoking is also discussed.
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Affiliation(s)
- S R Desai
- Department of Radiology, King's College Hospital, London, UK.
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Rajagopol J, Mark EJ. Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 32-2002. A 58-year-old man with interstitial pulmonary disease. N Engl J Med 2002; 347:1262-8. [PMID: 12393825 DOI: 10.1056/nejmcpc020013] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/19/2022]
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McClain KL, Gonzalez JM, Jonkers R, De Juli E, Egeler M. Need for a cooperative study: Pulmonary Langerhans cell histiocytosis and its management in adults. MEDICAL AND PEDIATRIC ONCOLOGY 2002; 39:35-9. [PMID: 12116077 DOI: 10.1002/mpo.10064] [Citation(s) in RCA: 17] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
BACKGROUND Pulmonary involvement with Langerhans cell histiocytosis (LCH, formerly known as histiocytosis-X) presents as an interstitial process in children and adults either with or without symptoms. In contrast to other manifestations of LCH, most patients with pulmonary disease are adults. PROCEDURES We reviewed the literature on pulmonary LCH to determine what were the clinical presentations, prognostic variables, and treatment options for this disease. RESULTS Although there are spontaneous remissions, a large number of patients have progressive pulmonary deficiency and experience significant morbidity if not mortality from the disease. The efficacy of steroid versus chemotherapy in halting the process remains controversial, even if smoking is taken into consideration. CONCLUSIONS A multicenter study of therapy for pulmonary LCH is the obvious answer to this dilemma. We propose that interested centers organize via the Histiocyte Society to plan and execute such a trial.
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Affiliation(s)
- Kenneth L McClain
- Baylor College of Medicine and Texas Children's Cancer Center and Hematology Service, Houston, Texas 77030, USA.
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Alhamad EH, Lynch JP, Martinez FJ. Pulmonary function tests in interstitial lung disease: what role do they have? Clin Chest Med 2001; 22:715-50, ix. [PMID: 11787661 DOI: 10.1016/s0272-5231(05)70062-9] [Citation(s) in RCA: 38] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/15/2023]
Abstract
Pulmonary function tests have been widely accepted and utilized in the management of interstitial lung diseases. Although the tests performed have changed little over the past several decades, extensive literature has been published highlighting their clinical role in the diagnosis, staging, prognostication, and follow-up of patients with a wide variety of interstitial lung diseases.
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Affiliation(s)
- E H Alhamad
- Division of Pulmonary and Critical Care Medicine, University of Michigan Medical Center, Ann Arbor, USA
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Akçay S, Eyüboglu FO, Arícan A, Demírhan B. Effect of pulse steroid therapy in a patient with Langerhans' cell histiocytosis. Respirology 2001; 6:357-60. [PMID: 11844129 DOI: 10.1046/j.1440-1843.2001.00353.x] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
Langerhans' cell histiocytosis (LCH) is a condition in which granulomas form in various tissues through the accumulation of abnormal histiocytes (Langerhans' cells), granulocytes and lymphocytes. An important distinction between LCH and primary pulmonary histiocytosis (PPH) is that smoking cessation is known to often lead to spontaneous improvement in PPH patients, while this has not been demonstrated in patients with multisystem LCH. In this case report, we describe the case of a 20-year-old man who presented with cough and palpably enlarged lymph nodes in his neck. An inguinal lymph node biopsy led to the diagnosis of multisystem LCH in the lungs, the cervical, intra-abdominal, and inguinal lymph nodes and the spleen. The patient was a smoker, and had averaged 30 cigarettes per day for 7 years. To address the multisystem involvement, intravenous pulse steroid therapy was administered (30-40 mg/kg daily) in two 3-day treatment periods separated by 3-4 weeks. The patient was also advised to stop smoking, and did so. Serial follow-up examinations confirmed an objective improvement with complete remission. The patient was still healthy at 18 months post-treatment, reflecting a dramatic and positive response to pulse steroid therapy. This encouraging result makes this case an important one to highlight.
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Affiliation(s)
- S Akçay
- Department of Pulmonary Diseases, Başkent University Hospital, Fevzi Cakmak Bulvari 10 Sokak, No: 45 06490 Bahçelievler, Ankara, Turkey.
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Bernstrand C, Cederlund K, Sandstedt B, Ahström L, Lundell M, Dahlquist G, Henter JI. Pulmonary abnormalities at long-term follow-up of patients with Langerhans cell histiocytosis. MEDICAL AND PEDIATRIC ONCOLOGY 2001; 36:459-68. [PMID: 11260569 DOI: 10.1002/mpo.1110] [Citation(s) in RCA: 46] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
BACKGROUND In Langerhans cell histiocytosis (LCH) pulmonary involvement, which is often initially asymptomatic, may contribute to significant morbidity and mortality. To determine the long-term prognosis, a cross-sectional study was undertaken. PROCEDURE Forty-one patients with > or = 5 years follow-up after the diagnosis of LCH were interviewed and underwent physical examination, blood tests, a chest X-ray and a high-resolution CT (HRCT) of the lungs. All patients included had been referred to the Department of Pediatrics at the Karolinska Hospital in Stockholm between July 1962 and February 1990 (median follow-up 16 years). Biopsies from all patients were reviewed and confirmed to be consistent with LCH. Information on previous clinical features including treatment and the results of chest X-rays were also collected for risk factor analysis. RESULTS Radiographic abnormalities of the lungs (cysts and/or emphysema), found in 10/41 (24%) at follow-up, were classified into five groups according to the extent of the cysts. These patients had more often suffered from multisystem than from single-system disease (P = 0.01), were significantly older at diagnosis (P < 0.001), and had been more heavily treated with chemotherapy and/or radiotherapy. They were also more frequently smokers (P < 0.0001) and 7/10 (70%) had suffered lung involvement at diagnosis. At the time of diagnosis of the pulmonary involvement, 4/10 (40%) patients had respiratory symptoms, but only 2/10 (20%) had symptoms at follow-up. CONCLUSIONS Ten (24%) of the 41 patients had abnormal findings on radiological examination of the lungs at long-term follow-up and seven are or had been smokers. It is of great importance that patients with LCH be informed about smoking-related pulmonary morbidity. Prolonged monitoring of the lungs for smokers and patients with known pulmonary involvement is recommended.
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Affiliation(s)
- C Bernstrand
- Childhood Cancer Research Unit, Astrid Lindgren Childreń's Hospital, Karolinska Hospital, Karolinska Institute, Stockholm, Sweden.
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Ryu JH, Colby TV, Hartman TE, Vassallo R. Smoking-related interstitial lung diseases: a concise review. Eur Respir J 2001; 17:122-32. [PMID: 11307741 DOI: 10.1183/09031936.01.17101220] [Citation(s) in RCA: 171] [Impact Index Per Article: 7.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022]
Abstract
Interstitial lung diseases (also known as diffuse infiltrative lung diseases) are a heterogeneous group of parenchymal lung disorders of known or unknown cause. These disorders are usually associated with dyspnoea, diffuse lung infiltrates, and impaired gas exchange. The majority of interstitial lung diseases are of unknown cause. Known causes of interstitial lung disease include inhalation of organic and inorganic dusts as well as gases or fumes, drugs, radiation, and infections. This review summarizes the clinical, radiological, and histopathological features of four interstitial lung disorders that have been linked to smoking. These disorders include desquamative interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, pulmonary Langerhans' cell histiocytosis, and idiopathic pulmonary fibrosis. Available evidence suggests most cases of desquamative interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, and pulmonary Langerhans' cell histiocytosis are caused by cigarette smoking in susceptible individuals. Smoking cessation should be a main component in the initial therapeutic approach to smokers with these interstitial lung diseases. In addition, smoking appears to be a risk factor for the development of idiopathic pulmonary fibrosis.
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Affiliation(s)
- J H Ryu
- Division of Pulmonary and Critical Care Medicine, Mayo Clinic Rochester, MN, USA
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35
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Soler P, Bergeron A, Kambouchner M, Groussard O, Brauner M, Grenier P, Crestani B, Mal H, Tazi A, Battesti JP, Loiseau P, Valeyre D. Is high-resolution computed tomography a reliable tool to predict the histopathological activity of pulmonary Langerhans cell histiocytosis? Am J Respir Crit Care Med 2000; 162:264-70. [PMID: 10903252 DOI: 10.1164/ajrccm.162.1.9906010] [Citation(s) in RCA: 51] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022] Open
Abstract
High-resolution computed tomography (HRCT) has proved to be very useful in the diagnosis and follow-up of pulmonary Langerhans cell histiocytosis (PLCH), but the precise relationships between nodules and thin-wall cysts observed by HRCT, and granulomatous or cystic lesions present in lung tissue, remain to be established. The aim of this study was to compare quantitative data obtained by HRCT and those obtained by histopathological examination of corresponding lung tissue specimens in patients with biopsy-proven PLCH. The results demonstrated that the extent of nodular abnormalities was strongly correlated with the density of florid granulomatous lesions in lung tissue. A strong correlation was also found between the extent of cystic abnormalities and the density of cavitary lesions, but the latter included both still inflammatory cavitary granulomas and cicatricial fibrous cysts. Interestingly, small isolated florid granulomas were found in lung tissue from most patients with a predominant cystic CT scan pattern. Taken together, these results demonstrate that HRCT has to be considered with caution to evaluate the histopathological activity of PLCH. Patients presenting with predominant HRCT cystic abnormalities should benefit from a long-term follow-up. Because these patients are susceptible to developing severe respiratory insufficiency, they should also be considered for treatment as soon as an effective therapy for LCH is available.
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Affiliation(s)
- P Soler
- INSERM U 408, UFR Xavier Bichat, Paris, France; Fédération des Maladies Respiratoires et Thoraciques, Hôpital Avicenne, AP-HP, Bobigny, France
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36
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Affiliation(s)
- A Tazi
- Inserm U82, Faculté Xavier Bichat, Paris, France
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Mogulkoc N, Veral A, Bishop PW, Bayindir U, Pickering CA, Egan JJ. Pulmonary Langerhans' cell histiocytosis: radiologic resolution following smoking cessation. Chest 1999; 115:1452-5. [PMID: 10334170 DOI: 10.1378/chest.115.5.1452] [Citation(s) in RCA: 109] [Impact Index Per Article: 4.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/01/2022] Open
Abstract
We describe two patients with histologically proven pulmonary Langerhans' cell histiocytosis in whom radiologic improvement occurred following smoking cessation. The patients had 23- and 25-pack-year smoking histories, respectively. High-resolution CT revealed multiple small nodules, located predominantly in the upper and middle lung fields. There was a close temporal relationship between smoking cessation and radiologic improvement.
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