Pulmonary langerhans cell histiocytosis springerlink. About 1 in 200,000 people get langerhans cell histiocytosis each year. Plch is characterized by bronchiolocentric nodules andor cysts in an upper and mid lung distribution with sparing of the costophrenic angles. Get my notes in pdf files that are downloadable at s. 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 orphanet journal of rare. Pulmonary langerhans cell histiocytosis plch is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. Pulmonary langerhans cell histiocytosis lch has been with us for over fifty years. Journal of the copd foundation alpha1 antitrypsin deficiency. Morphometric study of pulmonary arterial changes in. Pulmonary langerhans cell histiocytosis lch has been little has been added to.
Pulmonary langerhans cell histiocytosis stephen c juvet md1, david hwang md phd2, gregory p downey md3 1division of respirology and clinicianscientist training program, department of medicine. Pulmonary langerhans cell histiocytosis in a heavy marijuana smoker s. 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. 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. 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. Pulmonary langerhans cell histiocytosis radiology case. 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. Aberrant chemokine receptor expression and chemokine production by langerhans cells underlies the pathogenesis of langerhans cell histiocytosis. In adults, plch is frequently isolated and affects young smokers of both sexes. Plch can be one manifestation of langerhans cell histiocytosis, a multisystem disease.
Pulmonary langerhans cell histiocytosis plch restrictive. Pulmonary langerhans cell histiocytosis plch, pulmonary eosinophilic granuloma is a rare disease of clonal dendritic cells that primarily. Jci insight mapk mutations and cigarette smoke promote. The symptoms experienced by patients depend on the organs that are affected 1, 2. Infiltrate decreases, ultimately leading to scars with few or no langerhans cells. Define the imaging features of pulmonary langerhans cell histiocytosis. Langerhans cell histiocytosis is a type of cancer that can damage tissue or cause lesions to form in one or more places in the body. Chest xrays show nodules, small thickwalled lung cysts, and other changes that are typical of pulmonary langerhans cell histiocytosis. Current understanding and management of pulmonary langerhans. Recurrent nras mutations in pulmonary langerhans cell histiocytosis. The prognosis for children is highly variable, with infants and young children more likely to have systemic disease that leads to death. Langerhans cell histiocytosis lch belongs to a group of rare histiocytic. Pulmonary langerhans cell histiocytosis definition of.
In adults, by contrast, the lung is the most common and usually the sole organ affected. Pulmonary langerhans cell histiocytosis orphanet journal of. Proliferative disease or disorder of langerhans cells involving lung, highly associated with smoking pulmonary langerhans cell histiocytosis plch is thought to be distinct from systemic langerhans cell histiocytosis lch most common pulmonary histiocytic lesion arch pathol lab. Most cases affect children between 1 and 15 years old, with the majority of new cases in children between 5 and 10. Pulmonary langerhans cell histiocytosis plch is a diffuse lung disease that usually affects young adult smokers. Pulmonary langerhans cell histiocytosis plch is a smokingrelated disease of an undetermined etiology that predominantly affects young adults 14.
Pulmonary langerhans cell histiocytosis msd manual consumer. Newlyappearing lung nodules on surveillance imaging in patients with preexisting lung cancer can present a diagnostic dilemma when. Pulmonary langerhans cell histiocytosis masquerading as. Proliferative disease or disorder of langerhans cells involving lung, highly associated with smoking pulmonary langerhans cell histiocytosis plch is thought to be distinct from systemic langerhans cell histiocytosis lch most common pulmonary histiocytic lesion arch pathol lab med 2008.
Langerhans cell histiocytosis lch, also designated as histiocytosisx or eosinophilic granuloma, is an uncommon dendritic cell disorder characterized by the. A randomized trial of treatment for multisystem langerhans cell histiocytosis. Pulmonary langerhans cell histiocytosis plch is an uncommon but important cause of interstitial lung disease, and it occurs predominantly in adult cigarette smokers 1. Analyses of plch tissues have identified activating mutations of specific mitogenactivated protein kinases. 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. Cd1apositive langerhans cells produce inflammatory chemokines that are responsible for their own recruitment and retention. Langerhans cell histiocytosis lch is a rare disease marked by proliferation of langerhans type cells that share immunophenotypic and ultrastructural similarities with antigenpresenting langerhans cells of mucosal sites and skin. Initial and final ct scans of 21 patients with lch and ct evidence of pulmonary disease were compared retrospectively. Langerhans cells, which help regulate the immune system, are normally found throughout the body, especially in the skin.
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. Most mutations activate signaling enzymes that result in. It occurs predominantly in young smokers, without gender predominance. Langerhans cell histiocytosis blood american society.
The chemokine ccl20mip3alpha is responsible for t cell recruitment in lesions. Feline pulmonary langerhans cell histiocytosis with. Pulmonary langerhans cell histiocytosis is a rare granulomatous disease affecting both sexes, with greater incidence in the second and third decades of life. Nonetheless, the authors are successful in combining serial microscopic. Case discussion pulmonary lch has variable appearances but because there was cavitation of some of the nodules, lch was included in the imaging differential diagnosis. Adult pulmonary langerhans cell histiocytosis is a rare disorder of unknown aetiology that occurs predominantly in young smokers, with an incidence peak at 2040 yrs of age. Langerhans cell histiocytosis prognosis bmj best practice. List the pathologic features of pulmonary langerhans cell histiocytosis and address the difficulties and controversies related to its histologic diagnosis. Langerhans cell histiocytosis genetic and rare diseases. It is now considered a form of smokingrelated interstitial lung disease.
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. Pulmonary langerhans cell histiocytosis mayo clinic. Pulmonary langerhans cell histiocytosis pulmonary disorders. Er stress and basement membrane defects combine to cause glomerular and. 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. Some scholars conclude that combine hrct with the context of the. Simultaneous bilateral spontaneous pneumothorax in an adult patient. Pulmonary langerhans cell histiocytosis associated with. Smokingrelated interstitial pneumonias and pulmonary. There is much overlap on this article with histiocyte. It is regarded as a clonal accumulation and proliferation of abnormal bone marrow derived langerhans cells. Southwest journal of pulmonary and critical care2017volume 15 55 correct.
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. The lch cells in the lung and left neck lymph node were characterized by large. Approximately 50% of patients with plch harbor somatic brafv600e mutations in cells of the myeloidmonocyte lineage. Lch in adults diagnosis and treatment histiocytosis. Pulmonary hypertension ph is a common complication. A diagnosis of langerhans cell histiocytosis lch is made following a biopsy and microscopic examination of the affected tissue. It is caused by a disorder of myeloid dendritic cells. Review open access pulmonary langerhans cell histiocytosis.
Genomic alterations in langerhans cell histiocytosis. The term pulmonary langerhans cell histiocytosis was first described by farinacci in 1951 and refers to disease in adults that affects the lungs, either in isolation or in addition to other organ systems 1. Histiocytosis and langerhans cell histiocytosis should reference each other mccaul. Pulmonary langerhans cell histiocytosis with cervical lymph node. Medical management of langerhans cell histiocytosis from.
Pulmonary langerhans cell histiocytosis plch is a rare histiocytic disorder that almost exclusively affects the lungs of smokers. 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. Langerhanscell histiocytosis insight into dc biology. Mar 19, 2012 pulmonary langerhans cell histiocytosis plch is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. Langerhans cell histiocytosis etiology bmj best practice.
Smokinginduced lung diseases constitute a complex group of. Family history of cancer or having a parent who was exposed to certain chemicals may increase the risk of lch. Langerhans cell histiocytosis msd manual professional edition. Pulmonary langerhans cell histiocytosis radiology reference. People with plch experience breathing impairment that may range from mild to severe.
Pdf pulmonary langerhans cell histiocytosis mongwei. May 01, 2004 discuss the clinical presentation of patients with pulmonary langerhans cell histiocytosis. Pulmonary lch is nonclonal and, in many cases, appears to be a reactive. Most mutations activate signaling enzymes that result in extracellularsignalregulated kinase erk activation. These dendritic cells along with lymphocytes, eosinophils and normal histiocytes form infiltrates typical for the disease which may be found in various. 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. Pulmonary langerhans cell histiocytosis lch is a form of langerhans cell disease that primarily affects smokers in the third to fifth decade. Pulmonary langerhans cell histiocytosis request pdf. Dec 28, 2018 lch is relatively more common in children than in adults. Computed tomography ct may show these changes in enough detail to establish the diagnosis. In adults, pulmonary involvement with langerhans cell histiocytosis usually occurs as a singlesystem disease and is characterised by focal langerhans cell granulomas infiltrating and destroying distal. Arising from the aberrant accumulation of langerhans and other immune cells, plch tends to cause a relatively isolated pulmonary involvement as.
Langerhans cell histiocytosis lch braf map2k1 mek1 key points recurrent somatic genomic abnormalities occur in langerhans cell histiocytosis lch, indicating that it is a neoplastic disease. Pulmonary langerhans cell histiocytosis plch, also called eosinophilic granuloma of the lung, pulmonary langerhans cell granulomatosis, or pulmonary histiocytosis x, is an interstitial lung disease that occurs most often in young adults. The full text of this article is available as a pdf 530k. Langerhans cell histiocytosis is suspected in patients particularly young patients with unexplained pulmonary infiltrates, bone lesions, or ocular or craniofacial abnormalities. Symptoms are dyspnea, cough, fatigue, and pleuritic chest pain. While it may affect any organ of the body, few cases of solitary lung involvement are published in the literature. 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. Adult pulmonary langerhans cell histiocytosis european. Langerhans cell histiocytosis lch is a rare disease that may affect any age group. Simultaneous bilateral spontaneous pneumothorax in an adult. Etiology is unknown, but cigarette smoking plays a primary role. Plch is one of langerhans cell histiocytosis lch diseases that are.
Langerhans cell histiocytosis lch and lymphangioleiomyomatosis lam. Adults with pulmonary histiocytosis x have a poor prognosis. How i manage pulmonary langerhans cell histiocytosis. Langerhans cell histiocytosis lch is a rare disease of unknown aetiology. Pulmonary langerhans cell histiocytosis plch is proliferation of monoclonal langerhans cells in lung interstitium and airspaces. Lch cells are arrested in an immature, partially activated stage and show a deviant regulation of cell division. Pulmonary function testing shows that the amount of air the lungs can hold is below normal.
The disease is characterised by formation of eosinophilic. Pathologic findings were consistent with pulmonary langerhans cell histiocytosis lch. Pulmonary langerhans cell histiocytosis plch is a rare, smokingrelated, interstitial lung disease, and pulmonary hypertension ph is associated with mortality. Plch belongs to the spectrum of langerhans cell histiocytosis lch, diseases characterized by uncontrolled proliferation and infil. Sundar km, gosselin mv, chung hl, cahill bc 2003 pulmonary langerhans cell histiocytosis. The medical condition known as langerhans cell histiocytosis is a rare disorder that may cause damage to other organs of the body. Pulmonary langerhans cell histiocytosis plch is an uncommon disorder of adult smokers associated with a significant morbidity. Histiocytosis is a general name for a group of syndromes where immune cells known as histiocytes monocytesmacrophages proliferate and mistakenly attack the body instead of infections. Development of pulmonary langerhans cell histiocytosis in a patient.
Langerhans cell histiocytosis is a disorder in which excess immune system cells called langerhans cells build up in the body. 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. Pulmonary langerhans cell histiocytosis plch is a rare smokingrelated lung disease characterized by dendritic cell dc accumulation, bronchiolocentric nodule formation, and cystic lung remodeling. While the overwhelming majority of patients are smokers, mechanisms by. Pulmonary langerhans cell histiocytosis libre pathology. Here we describe 3 cats 2 males and 1 female with pulmonary langerhans cell histiocytosis plch. Here, we report a rare case of pulmonary lch plch in an adult. Dapi blue, anticd1a red and antinrasq61r antibody green, and a merged image of all three stains. Pulmonary langerhans cell histiocytosis plch is an interstitial lung disease ild occurring nearly exclusively in smokers. Langerhans cell histiocytosis lch is the unifying designation for a rare proliferative disorder. Pulmonary langerhans cell histiocytosis forms part of a spectrum of diseases characterized by monoclonal proliferation and infiltration of organs by langerhans cells table 1. Langerhans cell histiocytosis genetics home reference nih. Pulmonary langerhans cell histiocytosis has a variable appearance depending on the stage of disease, ranging from small peribronchiolar nodular opacities to.
We herein report a case of plch complicated by severe ph and respiratory impairment. They compared and contrasted the clinical, radiologic, and histologic characteristics of various causes of advanced fibrotic ild, including connective tissue diseaseassociated ild, chronic hypersensitivity pneumonitis, advanced pulmonary langerhans cell histiocytosis, endstage pulmonary sarcoidosis, erdheimchester disease, and hermansky. Pulmonary langerhans cell histiocytosis chest foundation. Pulmonary langerhans cell histiocytosis plch is rare in childhood but occurs most commonly in children with multisystem ms lch. Search for articles by this author, first page pdf related. The signs and symptoms of lch depend on where it is in. Plch is characterized by peribronchiolar infiltration by langerhans and inflammatory cells and formation of granulomas, leading to stellate interstitial nodules 22, 29.
The prognostic value of a response to treatment assessed at 6 weeks has also been validated, in that if the patient responds to treatment by 6 weeks, they have a better prognosis. Pulmonary langerhans cell histiocytosis genetic landscape includes recurrent. Pulmonary langerhans cell histiocytosis surgical pathology. The disease can regress spontaneously after smoking cessation, but it sometimes progresses to chronic respiratory insufficiency 57. 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. Its clinical course is typically characterized by stabilization or regression of bilateral micronodular infiltrates seen on chest radiographs. Langerhans cell histiocytosis lch is caused by an uncontrolled pathogenic clonal proliferation of dendritic cells dcs with langerhans cell lc characteristics. Sep 11, 2017 langerhans cell histiocytosis lch is a disorder that primarily affects children, but is also found in adults of all ages. If the biopsy is positive for lch, some other tests may be carried out to determine the extent of disease so that a treatment plan can be made. Pulmonary langerhans cell histiocytosis plch is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette. Pulmonary langerhans cell histiocytosis in a heavy. Plch affects adults of all backgrounds, but the disease almost exclusively occurs in cigarette smokers. Pulmonary langerhans cell histiocytosis histiocytosis x on bronchoalveolar lavage.
No related items previous abstract next abstract 25 broadway. Pulmonary langerhans cell histiocytosis plch is an unusual cystic lung disease that is also characterized by extrapulmonary manifestations. Pulmonary langerhans cell histiocytosis plch is a rare and serious lung disease that occurs in adults. The term pulmonary langerhanscell histiocytosis is used to refer to disease in adults that affects the lung, either in isolation or in addition to other organ systems. Pulmonary langerhans cell histiocytosis orphanet journal. The pathologic hallmark of plch is the accumulation of langerhans and other inflammatory cells in small. A stepwise diagnostic approach to cystic lung diseases for. To document the evolution of pulmonary lesions of langerhans cell histiocytosis lch with sequential computed tomography ct. Pulmonary langerhans cell histiocytosis plch is a rare interstitial lung disease that typically affects young adults and is associated with cigarette smoking 20,22. Reduced exercise capacity, due to a blend of pseudorestriction, air. Pulmonary langerhans cell histiocytosis plch is a relatively rare disease. The cats were euthanized due to progressive respiratory clinical symptoms and deterioration. Pulmonary langerhans cell histiocytosis radiographics.
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