Corticosteroids is the preferred treatment for this form of lch. The definite diagnosis of pulmonary langerhans cell histiocytosis requires identification of langerhans cell. Pulmonary langerhans cell histiocytosis associated pulmonary. Langerhans cell histiocytosis lch describes a spectrum of clinical presentations ranging from a single bone lesion or trivial skin rash to an explosive disseminated disease.
Medical management of langerhans cell histiocytosis from. Langerhans cell histiocytosishealth professional version. Malignant histiocytosis syndrome and hemophagocytic syndromes are the two other classes of histiocytosis. Some individuals may even present with sudden shortness of breath associated with spontaneous lung collapse or recurrent lung infections. How i manage pulmonary langerhans cell histiocytosis. Pulmonary langerhans cell histiocytosis has variable appearance depending on the stage of disease, ranging from small peribronchiolar nodular opacities to multiple irregularlyshaped cysts. Pulmonary langerhans cell histiocytosis plch is an uncommon but important cause of interstitial lung disease, and it occurs predominantly in adult cigarette smokers. Langerhans cell histiocytosis lch is a rare disease, which occurs mainly in children.
Arising from the aberrant accumulation of langerhans and other immune cells, plch tends to cause a relatively isolated pulmonary involvement as compared to other forms of langerhans cell lc and histiocytic disorders. Langerhans cell histiocytosis nanette grana, md background. Pulmonary langerhans cell histiocytosis plch is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. Pdf pulmonary langerhans cell histiocytosis plch is a rare disease with insidious onset and nonspecific manifestations. The term pulmonary langerhans cell histiocytosis is used to refer to disease in adults that affects the lung, either in isolation or in addition to other organ systems. About 15% of patients are asymptomatic, with disease noted incidentally on a chest xray taken for another reason. Pulmonary langerhans cell histiocytosis plch is a rare disorder of unknown cause characterised by the infiltration of the lungs and other organs by the bone marrow derived langerhans cells, which carry mutations of braf gene andor nras, kras and map2k1 genes.
Pulmonary langerhans cell histiocytosis is an uncommon smokingrelated lung disease it is also known as eosinophilic granuloma of the lung the term langerhans cell histiocytosis refers to several different diseases. Langerhans cell histiocytosis is a rare, multisystemic pathology that can affect many organs, including bones and lungs and largely affecting females, with a higher prevalence in smokers. Pulmonary langerhans cell histiocytosis radiographics. Gastrointestinal involvement in langerhans cell histiocytosis. This article includes a pictorial essay of typical. Differential diagnosis for manifestations of langerhans cell histiocytosis. 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. Pulmonary langerhans cell histiocytosis the american journal of. Plch belongs to the spectrum of langerhans cell histiocytosis lch, diseases characterized by uncontrolled proliferation and infil. Current understanding and management of pulmonary langerhans. Pulmonary langerhans cell histiocytosis request pdf. Pulmonary langerhans cell histiocytosis plch is a rare interstitial granulomatous disease that usually affects young adults who are smokers. Pulmonary langerhans cell histiocytosis archives of pathology.
Langerhans cell histiocytosis lch in adults is a rare disorder that occurs when the body produces too many langerhans cells histiocytes, which are a type of white blood cell that helps fight infection. Srj is a prestige metric based on the idea that not all citations are the same. Lch is an abnormal proliferation and dissemination of clonal langerhans cells in which they accumulate, along with other inflammatory cells, and form tissue granulomas in different organs. Langerhans cell histiocytosis lch in adults is a rare disorder that occurs when the body produces too many langerhans cells histiocytes, which are a. It is caused by a disorder of myeloid dendritic cells. Langerhans cell histiocytosis lch or histiocytosis x is the primary form of the condition, although there are a variety of related syndromes. In this study, the records of 102 adults with clearly. Pulmonary langerhans cell histiocytosis is a disease in which monoclonal cd1apositive langerhans cells a type of histiocyte infiltrate the bronchioles and alveolar interstitium, accompanied by lymphocytes, plasma cells, neutrophils, and eosinophils. Pulmonary langerhans cell histiocytosis is a chronic lung disease that presents with none or mild symptoms, such as shortness of breath or cough in early disease. Get detailed treatment information for lch in this summary for clinicians. Its its clinical presentation is variable and ranges from isolated skin or bone disease to a lifethreatening multisystem. It occurs predominantly in young smokers, without gender predominance. Open biopsy for chronic diffuse infiltration lung disease.
Pulmonary langerhans cell histiocytosis presenting as a. Langerhans cell histiocytosis genetic and rare diseases. Pulmonary langerhans cell histiocytosis associated pulmonary hypertension report of two cases case report abstrac t langerhans cell histiocytosis lch is a multisystemic disease affect ing mainly the skeleton and the lungs. This disorder is characterized by an abnormal increase in certain immune cells called histiocyte cells. Pulmonary langerhans cell histiocytosis plch can be seen as part of widespread involvement in patients with disseminated lch or more frequently as a.
Review open access pulmonary langerhans cell histiocytosis harpreet s suri1, eunhee s yi2, gregorz s nowakowski3 and robert vassallo1,4 abstract pulmonary langerhans cell histiocytosis plch is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. Pathologic findings were consistent with pulmonary langerhans cell histiocytosis lch. Langerhans cell histiocytosis lch treatment may include observation alone, surgery, radiation therapy, or oral, topical, and intravenous medication. Pulmonary langerhans cell histiocytosis plch may be seen as part of widespread involvement in patients with disseminated lch or more frequently as a distinct entity in young adult smokers. Etiology is unknown, but cigarette smoking plays a primary role. A biopsy of the lesions showed that these lesions were lngerhans with langerhans cell histiocytosis. Pulmonary langerhans cell histiocytosisassociated pulmonary hypertension report of two cases case report abstrac t langerhans cell histiocytosis lch is a multisystemic disease affect ing mainly the skeleton and the lungs. Pulmonary eosinophilic granuloma, also known as langerhans cell granulomatosis, langerhans cell histiocytosis, and histiocytosis x, is an interstitial lung disease characterized by an abnormal proliferation of langerhans cells. Approximately 1015% of cases also involve other sites primarily bone.
Men are more commonly affected than women, by a ratio of 1. Pulmonary langerhans cell histiocytosis plch is a diffuse lung disease. These disorders are thought to result from the proliferation of specific histiocytic cells, known as langerhans cells, and their infiltration of organ systems. Langerhans cell histiocytosis is a rare systemic disease. Involvement manifestation possible other condition skin vesicles and bullae most common in early infancy erythema toxicum herpes simplex chickenpox infection dermatitis most frequently scalp, diaper or axilla, may occur up to late infancy seborrheic dermatitis. Langerhanscell histiocytosis a spectrum of diseases formerly known as histiocytosis x and now called langerhanscell histiocytosis is characterized by erk pathway activation including braf v600e. Utility of bronchoscopy in pulmonary langerhans cell. Pulmonary langerhans cell histiocytosis plch is an uncommon disorder of adult smokers associated with a significant morbidity.
How i treat langerhans cell histiocytosis blood american. Feb 28, 2017 langerhans cell histiocytosis lch is one of the histiocytosis disorders, as defined by the histiocyte society. For patients who have pulmonary langerhans cell histiocytosis, the most part of the treatment is the cessation of cigarette smoking and this will improve the symptoms in some patients. Sep 11, 2017 langerhans cell histiocytosis lch is a disorder that primarily affects children, but is also found in adults of all ages. Pulmonary langerhans cell histiocytosis lch manifests with dyspnoea and a cough with no significant expectoration. Pulmonary langerhans cell histiocytosis radiology case. Lch may affect patients of all ages, ranging from neonates to the elderly 2, 3. Pulmonary langerhans cell histiocytosis chest foundation. A flow cytometric study of langerhans cell histiocytosis. Pulmonary langerhans cell histiocytosis is a form of interstitial lung disease ild that is thought to be distinct from systemic lch table 1. Pulmonary langerhans cell histiocytosis orphanet journal of.
Increased numbers of langerhans cells are found in other lung diseases that afflict smokers, including chronic obstructive pulmonary disease. Review open access pulmonary langerhans cell histiocytosis. Pulmonary langerhans cell histiocytosis libre pathology. Ten percent to 25% of patients have sudden, spontaneous pneumothorax. Pulmonary langerhans cell histiocytosis springerlink. Case 10 case an immunemediated mechanism has been postulated, although an inciting agent has not been isolated 4.
These dendritic cells along with lymphocytes, eosinophils and normal histiocytes form infiltrates typical for the disease which may be found in various. The natural history of plch is unclear due to a lack of prospective studies. Other symptoms include constitutional symptoms fatigue and weight losspleuritic chest pain, or spontaneous pneumothorax pulmoonar. Langerhans cell histiocytosis lch is a rare cancer involving clonal proliferation of langerhans cells, abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes. Aug 21, 2019 langerhans cell histiocytosis micrograph showing a langerhans cell histiocytosis with the characteristic reniform langerhans cells accompanied by abundant eosinophils. In adults, pulmonary involvement with langerhans cell histiocytosis usually occurs as a singleorgan disease and is characterized by focal langerhans cell granulomas infiltrating and destroying distal bronchioles. Histiocytic diseases, like langerhans cell histiocytosis lch and myeloidderived dendritic cell disorder, in children and adults are caused by an abnormal accumulation of cells of the mononuclear phagocytic system. Find evidencebased information on langerhans cell histiocytosis treatment. Langerhans cell histiocytosis lch is a rare disorder of unknown aetiology that is characterised by the infiltration of involved tissues by dendritic cells sharing phenotypic similarities with langerhans cells, which are often organised into granulomas 1. Pulmonary langerhans cell histiocytosis plch is an uncommon but important cause of interstitial lung disease, and it occurs predominantly in adult cigarette. Pathogenesis of plch appears to involve recruitment, activation, and retention of langerhans cells in a bronchiolocentric pattern, a process likely triggered by exposure to constituents of cigarette smoke. Pulmonary langerhans cell histiocytosis pulmonary disorders.
Adult langerhans cell histiocytosis with pulmonary and colorectoanal involvement. Fulltext pdf histiocytosis x and acute monocytic leukemia. Pulmonary langerhans cell histiocytosis plch is proliferation of monoclonal langerhans cells in lung interstitium and airspaces. Langerhans cell histiocytosis lch is a rare histiocytic disorder of unknown etiopathogenesis. It is an uncommon interstitial lung disease whose radiological findings are characterized by cen. 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. Pulmonary langerhans cell histiocytosis plch is a rare cystic disorder of unknown origin that occurs in young adult smokers. Clinical outcomes of pulmonary langerhanscell histiocytosis. Continuing navigation will be considered as acceptance of this use. Langerhans cell histiocytosis blood american society of. Lch is part of a group of syndromes called histiocytoses, which are characterized by an abnormal proliferation of histiocytes an. Langerhans cell histiocytosis is a systemic disorder, meaning it affects the entire body. Langerhans cell histiocytosis a spectrum of diseases formerly known as histiocytosis x and now called langerhans cell histiocytosis is characterized by erk pathway activation including braf v600e.
Langerhans cell histiocytosis blood american society. Includes all cases in which the lung is the principal site of involvement. While the overwhelming majority of patients are smokers, mechanisms by. Pulmonary langerhans cell histiocytosis plch is a rare disease with insidious onset and nonspecific manifestations. Pulmonary langerhans cell histiocytosis plch, previously called eosinophilic granuloma of the lung, pulmonary langerhans cell granulomatosis, and pulmonary histiocytosis x, is an uncommon cystic interstitial lung disease that primarily affects young adults. Diagnosis is based on history and imaging tests and sometimes on bronchoalveolar. Pulmonary langerhans cell histiocytosis associated. Pulmonary langerhans cell histiocytosis is an uncommon interstitial lung disease that results from the accumulation of cells known as langerhans cells in the lung. Langerhans cell histiocytosis also called histiocytosis x is a rare disorder that primarily affects children. People with lch produce too many langerhans cells or histiocytes, a form of white blood cell found in healthy people that is supposed to protect the body from infection.
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. Langerhans cell histiocytosis micrograph showing a langerhans cell histiocytosis with the characteristic reniform langerhans cells accompanied by abundant eosinophils. Pulmonary langerhans cell histiocytosis plch is an unusual cystic lung disease that is also characterized by extrapulmonary manifestations. Pulmonary langerhans cell histiocytosis orphanet journal. It is regarded as a clonal accumulation and proliferation of abnormal bone marrow derived langerhans cells. Symptoms are dyspnea, cough, fatigue, and pleuritic chest pain. The pathologic hallmark of plch is the accumulation of langerhans and other inflammatory cells in small airways, resulting in the formation of.
Frequently children affected are between years old and boys are more often affected. Pulmonary langerhans cell histiocytosis plch is an uncommon form of interstitial lung disease that is usually associated with smoking when occurring in adults. Pulmonary langerhans cell histiocytosis plch is an uncommon but important cause of interstitial lung disease, and it occurs predominantly in adult cigarette smokers 1. Adult pulmonary langerhans cell histiocytosis european. Treatment depends on the site and extent of disease. While the overwhelming majority of patients are smokers, mechanisms. The disease is characterised by formation of eosinophilic.
Pulmonary langerhans cell histiocytosis in adults is a rare interstitial lung disease, and its course and outcome have not been well defined. Plch belongs to the spectrum of langerhans cell histiocytosis lch, diseases characterized by uncontrolled proliferation and infiltration of various organs by langerhans cells. Pulmonary langerhans cell histiocytosis mayo clinic. In the present case, the symptomatology began at a skeletal level, but showing some pulmonary findings compatible with an early stage of pulmonary involvement. Symptoms range from isolated bone lesions to multisystem disease. Histiocyte cells are a form of white blood cells that help the immune system destroy foreign materials and fight infection. Adult langerhans cell histiocytosis with pulmonary and. It is reported to be between 26 54 cases per million children in the general population.
The pathogenic cells are defined by constitutive activation of the mapk signaling pathway. Pulmonary langerhans cell histiocytosis radiology reference. The natural history of adult pulmonary langerhans cell. A flow cytometric fcm study of a single case has, however, been published which claimed to provide evidence to contradict this. Langerhans cell histiocytosis lch, or histiocytosis x, is now generally considered to be a nonmalignant condition. Langerhans cell histiocytosis lch is a rare disease that may affect any age group. Typical symptoms and signs of pulmonary langerhans cell histiocytosis are dyspnea, nonproductive cough, fatigue, fever, weight loss, and pleuritic chest pain.
The disease can resolve spontaneously, remain stable, or progress to respiratory failure with severe pulmonary hypertension ph, requiring lung transplantation 1,2,4. Discussion dendritic cells are components of the mammalian immune system that are chiefly located in tissues that contact the external environment, such as the skin, lungs, and gastrointestinal tract. Pathogenesis of plch appears to involve recruitment, activation, and retention of langerhans cells in a bronchiolocentric pattern, a process likely triggered by exposure to constituents of cigaret. Pulmonary langerhans cell histiocytosis plch pulmonary langerhans cell histiocytosis plch is a unique form of lch in that it occurs almost exclusively in cigarette smokers. It is now considered a form of smokingrelated interstitial lung disease. In adults, plch is frequently isolated and affects young smokers of both sexes. Targeted therapy with braf inhibitors has been used in select patients with lch, and the results have been encouraging.
1006 1267 704 1077 129 1321 901 626 1541 957 733 429 1063 355 1121 451 1354 1094 416 179 129 1174 956 469 36 144 336 469 289 1111 322 1075 525 765 85 227 201 1425 280 997